TEXAS OVERVIEW OF STATE RESPONSE TO CHRONIC WASTING DISEASE LEGISLATIVE BUDGET BOARD STAFF – APRIL 2019 LEGISLATIVE BUDGET BOARD STAFF REPORTS – ID: 4830

Texas OVERVIEW OF STATE RESPONSE TO CHRONIC WASTING DISEASE

LEGISLATIVE BUDGET BOARD STAFF – APRIL 2019 LEGISLATIVE BUDGET BOARD STAFF REPORTS – ID: 4830

This overview, which was prepared at the request of members of the Legislature, shows the state’s response to chronic wasting disease, including agency authority, activities, and expenditures.

https://www.lbb.state.tx.us/Documents/Publications/Staff_Repor t/2019/4754_WildlifeDiseaseManagement.pdf

News Release Media Contact: Steve Lightfoot, 512-389-4701, steve.lightfoot@tpwd.texas.gov

Oct. 21, 2014

Pair Convicted in Illegal Deer Breeding Operation

AUSTIN – The latest chapter in a decade long series of criminal and wildlife disease investigations involving a former South Texas deer breeder ended recently when a Corpus Christi area couple pleaded guilty to 50 charges of Unlawful Possession and/or Sale of Live Game Animals.

Frank Thomas Shumate Jr., 51, and Kalub Rogers Shumate, 31, were each assessed $14,127.50 in fines and agreed to surrender the ability to apply for a deer breeder permit or a hunting lease license for all time. Mr. Shumate also agreed to surrender his hunting license through the end of the 2015 license year and Ms. Shumate through the end of the 2017 license year.

The criminal cases were adjudicated in the office of Hon. Caroline Korzekwa, Karnes County Justice Court Precinct. 2.

Retired San Antonio attorney Rene Barrientos served as special prosecutor in the case with approval and support from the Karnes County Attorney. He also coordinated a global agreement in Travis County District Court to resolve a pending civil case against Ms. Rogers and recover $34,080 in restitution related to expenses incurred by TPWD staff while conducting a deer herd inventory inspection and disease sampling at a deer breeding facility permitted to Ms. Rogers.

Investigation into Mr. Shumate’s deer breeding activities began in March 2004 and led to multiple charges in three counties. Two years later, his deer breeder violations resulted in 10 convictions on criminal charges in Jim Wells County, 5 convictions in Nueces County and 5 convictions in Webb County. As a result of these findings, Mr. Shumate agreed to relinquish his Scientific Breeder’s Permit and liquidate all deer held in captivity in his deer breeding facility in Nueces County.

In advance of losing his deer breeding privileges, Mr. Shumate allegedly initiated a plan to have a new deer breeding facility permitted in the name of Kalub Rogers on his property in Karnes County, where he then transferred more than 100 deer from his Nueces County facility.

Over time the TPWD Special Operations Unit received numerous reports that Mr. Shumate was reportedly still in the deer business and was buying and selling deer for which he was not legally authorized by TPWD. An investigation revealed that Mr. Shumate conducted sales of at least 78 white-tailed deer from Ms. Rogers’ deer breeding facility to ranches for release into the wild for stocking purposes since September 2010. Mr. Shumate received a minimum of $171,466 in payments for white-tailed deer he unlawfully sold, which according to records submitted to TPWD, were transported from Ms. Rogers’ deer breeding facility. The investigation further indicated that Kalub Rogers was holding a deer breeder permit in her name on behalf of her husband Frank Thomas Shumate Jr.

Ms. Rogers’ facility came under additional scrutiny in 2012, initially as a result of a delinquent annual report required of all permitted deer breeding facilities. Criminal charges were filed when a subsequent herd inspection and inventory revealed significant irregularities and discrepancies, including 162 inventoried deer that were missing from the facility. During the herd inspection, TPWD wildlife biologists noted the remaining deer in the facility to be in poor condition and numerous decayed deer carcasses were observed throughout the pens.

The observation of 142 deer of unknown origin was cause for additional concern and tissue samples from several deer were tested for both Chronic Wasting Disease and Bovine Tuberculosis. Neither disease was detected.

“Unscrupulous actions by these two individuals are not only a threat to all the law-abiding deer breeders who are carefully monitoring and managing their facilities, but also to the state’s free-ranging deer, which can be exposed to unnecessary disease risk from these illegitimate activities,” said Mitch Lockwood, TPWD Big Game Program Director. “Captive and free-ranging deer are too important to our state to have them compromised by the actions of a few.”

https://tpwd.texas.gov/newsmedia/releases/?req=20141021a

June 15, 2011

Cherokee County Deer Breeder Pleads Guilty to Smuggling Deer

Prominent breeder Agreed to Pay $1.5 million for Smuggling Deer into East Texas

TYLER – After a lengthy four year investigation a 77-year-old Cherokee County, Texas licensed deer breeder has pleaded guilty to illegally transporting wildlife in the Eastern District of Texas and then lying about it to a U.S. Fish and Wildlife agent, announced U.S. Attorney John M. Bales today.

Billy Powell pleaded guilty on June 14, 2011, to the felony offense of smuggling at least 37 whitetail deer, over a 3 year time span, from Indiana, Illinois, and Ohio into Texas in violation of state and federal laws. Powell also admitted that he made a false statement and submitted a false document to a U.S. Fish and Wildlife special agent who was looking into the matter. Powell has agreed to pay a $1 million fine, to be deposited into the U.S. Fish and Wildlife Lacy Act Reward Fund, as well as $500,000.00 in restitution to Texas Parks and Wildlife, on his sentencing date. Powell’s agreement with the government calls for Powell to serve 3 years probation with six months of home confinement which will be monitored with an electric anklet. During the term of probation, Powell will be prohibited from participating in any manner in commercial deer breeding. Additionally, Powell must forfeit any illegally imported deer, any progeny of those deer, and any biological material derived from said deer, which would include any semen, antlers, mounts, and cloned deer. Powell has already forfeited over 1,300 straws of frozen semen valued at approximately $961,500.00 to U.S. Fish and Wildlife.

According to information presented in court, on at least four separate occasions, spanning from October 2006 through June 2008, Powell knowingly imported at least 37 live whitetail deer, many of whom came from captive deer farms in Ligonier, Indiana, into the state of Texas and to his “5-P Farms”, high fenced deer breeding facility in Cherokee County Texas. These deer included bucks known as “Fat Boy” aka “Barry”, “Silver Storm” aka “Hit Man”, “Y 009", “Eagle Storm” aka “BJ”, “Thunderstruck”, “High Five”, and “Primer” aka “Spikes”. At all times Powell knew that Texas law prohibited any person from possessing a deer acquired from an out-of-state source. In spite of this, Powell agreed to participate in the above-described transactions in which whitetail deer would be secretly transported from Illinois, Indiana, and/or Pennsylvania, to Texas in order to evade Texas laws and regulations.

Powell acknowledged that the fair market value of all of the illegally imported, whitetail deer exceeded approximately $800,000.00, that the value of the illegally accumulated white-tailed deer semen exceeded approximately $961,000.00, and that the value of the progeny exceeded approximately $290,000.00.

Powell further admitted that he lied to a U.S. Fish and Wildlife Special Agent during a voluntary statement at the U.S. Attorney’s Office in Tyler, Texas. Powell told the agent that he had illegally imported approximately 35 white-tailed deer into the state of Texas when in fact he knew that he had illegally imported no less than 41 white-tailed deer, including 6 white-tail deer fawns. During the same statement, Powell also submitted lists identifying 35 white-tailed deer as the total number of white-tailed deer that he had illegally imported into the state of Texas when he knew that he had actually illegally imported no less than 41 white-tailed, including 6 white-tail deer fawns.

Findings of the investigation also prompted the Wildlife Division of Texas Parks and Wildlife Department to conduct an epidemiological investigation in consultation with veterinarians and wildlife disease experts from Texas Animal Health Commission, Texas Department of State Health Services, and Texas A&M College of Veterinary Medicine and accredited veterinarians actively involved in the deer breeding industry. This process was carried out in three separate phases. Ultimately all 334 deer contained in Powell’s deer breeding facility were euthanized to facilitate testing for chronic wasting disease (CWD) and bovine tuberculosis (TB). This process was necessary in order to provide an acceptable level of assurance that neither disease was prevalent in Powell’s deer breeding facility nor in any deer breeding facility that had received deer from Powell’s facility since October 2004.

Texas Parks and Wildlife Department has had an intensive CWD surveillance program since 2002, and this disease has yet to be detected in Texas. Likewise, bovine tuberculosis has not been detected in any Texas deer population. However, illegal entry of white-tailed deer from other states poses a serious risk of introducing these diseases and others into Texas. Introduction of these diseases into Texas could have a detrimental impact on the longtime cultural tradition of deer hunting, which generates an estimated $1.2 billion in retail sales and has a total economic output of more than $2 billion in Texas each year. Disease monitoring is also necessary to protect legal deer breeding activity from risk of disease exposure. Furthermore, bovine tuberculosis could have a significant impact on the Texas livestock industry. Prevention is the most effective tool to combat diseases because once established in wild populations, these diseases are extremely difficult, if not impossible to eradicate.

Since no live-animal test for CWD exists, TPWD consulted with trained experts to ensure the most humane euthanasia method and treatment of the animals was used. Texas Parks and Wildlife officials are presently awaiting the test results for the tissue samples submitted to the Texas Veterinarian Medical Diagnostic Laboratory located in College Station, TX

This case was investigated by the Special Operations Unit of the Texas Parks and Wildlife and U.S. Fish and Wildlife Service and prosecuted by Assistant U.S. Attorney Jim Noble.

https://tpwd.texas.gov/newsmedia/releases/?req=20110615a

Texas deer breeder sentenced for smuggling

Aug. 18, 2010 at 3:18 a.m.

TYLER, Texas (AP) - A licensed deer breeder from Austin must serve three years of probation for illegally transporting wildlife in east Texas.

U.S. Attorney John Bales says 41-year-old Lance Clawson was sentenced Wednesday in Tyler.

Clawson on April 14 pleaded guilty to smuggling whitetail deer from Oklahoma into Texas. He also was fined $15,000 and must pay $7,250 in restitution to the Texas Parks and Wildlife Foundation.

Investigators say Clawson, who operates a ranch and deer breeding facility near Goldthwaite, traveled to Muskogee, Okla., on Oct. 15, 2008, to buy fawns from a breeder.

Clawson's vehicle was stopped the following day by Texas game wardens as he returned with eight fawns.

Texas law bans importing live whitetail or mule deer due to the threat of transmittable diseases.

https://www.victoriaadvocate.com/news/2010/aug/18/bc-tx-deer-breeder-smuggling/

News Release Media Contact: Mike Cox, 512-389-8046, mike.cox@tpwd.texas.gov

Feb. 5, 2010

Texas Men Sentenced to Federal Prison for Deer Trafficking, Stolen Property

AUSTIN — A four-year, multi-agency investigation that began when two Texas Parks and Wildlife Department Game Wardens received information about possible stolen property on two Northeast Texas ranches has ended with federal prison sentences for a father and son who pled guilty to transporting stolen property and wildlife trafficking.

Forty-nine-year-old James Dwayne Anderton and his 26-year-old son Jimmie Wallace Anderton, both of Quinlan in Hunt County, Texas were sentenced Tuesday in U.S. District Court in Sherman by Judge Marcia Crone.

"This case is an excellent example of a joint effort on the part of local, state and federal law enforcement," said Col. Pete Flores, TPWD’s law enforcement director. "We’re proud of the work our Game Wardens put in on this case, but everyone involved did a great job in pursuing a complicated investigation that not only resulted in the recovery of thousands of dollars worth of stolen property, it will help keep our Texas deer herd safe from disease by ending an illegal importation operation."

The elder Anderton drew 30 months in federal prison for interstate transportation of stolen property and was ordered to pay $180,952 restitution. His son will serve 27 months in federal prison for interstate transportation and will be jointly responsible for making restitution. In addition, both men got 12 months federal confinement for violations of the Federal Lacey Act involving the illegal transportation of deer across state lines. These sentences will be served concurrently with their other sentence, but they also face 36 months of supervised release following their discharge from prison.

Benjamin Tuggle, Regional Director for the U.S. Fish and Wildlife Service, agrees with Col. Flores, adding, "We appreciate these kinds of collaborative efforts. It is all about wildlife conservation and to be successful we need to work together. All of the law enforcement agents working on this case are to be commended for their dedication and hard work."

The Andertons operate a turf grass and landscaping business and hold a TPWD permit to breed captive deer. In the spring of 2006 Game Wardens Dale Waters and Eric Collins were looking into some possible deer breeding violations when they received information that the Andertons might be in possession of stolen heavy equipment and trailers.

The wardens relayed this information to Texas Department of Public Safety Motor Vehicle Theft Division Sgts. John Murphy and Rex Wilemon, who drew up a search warrant executed on two pieces of land owned by the Andertons, one in Delta County and the other in Hunt County near Quinlan. Six Game Wardens and Sgt. Brad Chappell of the TPWD Special Operations Unit participated in the search, which resulted in the recovery of stolen farm machinery and construction equipment. Deputies with the Delta and Hunt county sheriff’s departments and members of the Northeast Texas Auto Theft Task Force also assisted in the search.

The two DPS investigators presented the findings from their stolen equipment investigation to FBI Special Agent Ken Paith who furthered the investigation and developed sufficient evidence to indict the Andertons on July 8, 2009 for interstate transportation of stolen property. Also indicted on the same charge was Timothy Shane Peavler, 37, of Lone Oak, Texas.

During this time Chappell received information that the Andertons had been unlawfully importing white-tailed deer from Arkansas. The Game Warden sergeant contacted U.S. Fish and Wildlife Service Special Agent Mike Merida and began a joint investigation along with Little Rock-based Fish and Wildlife Special Agent Glen Pye and investigators with Arkansas Fish and Game Commission.

This cooperative effort eventually revealed the source of the deer the Andertons were illegally importing. Merida and Chappell determined the Andertons had paid more than $63,000 for at least 125 white-tailed deer and imported them to Texas in violation of a state statute which restricts deer importation.

The two officers eventually learned that the Andertons had hired people to travel to a deer breeder’s facility in northern Arkansas, load deer and return to Texas where they delivered the illegal cargo to both the Anderton’s properties.

Peavler pled guilty on July 27 last year to interstate transportation of stolen property and was sentenced to six months in federal prison and ordered to pay $42,403 restitution. The Andertons pled guilty Aug. 31.

"TPWD began limiting importation of live white-tailed deer and mule deer into Texas in the spring of 2002 due to concerns over potential spread of two diseases that could be devastating to our deer population, chronic wasting disease (CWD) and bovine tuberculosis," Flores said. "We have worked hard to prevent CWD from being introduced into Texas and so far our state has remained CWD free."

Assistant United States Attorney Randall Blake prosecuted the case.

https://tpwd.texas.gov/newsmedia/releases/?req=20100205c

Appeal from the United States District Court for the Northern District of Texas No. 3:13-CV-01641

JAMES ANDERTON; JIMMIE ANDERTON,

Plaintiffs – Appellants

TEXAS PARKS AND WILDLIFE DEPARTMENT; TEXAS ANIMAL HEALTH COMMISSION; CARTER SMITH, Executive Director, Texas Parks and Wildlife Department; CRAIG HUNTER, Director, Texas Parks and Wildlife Department; L. DAVID SINCLAIR, Chief Game Warden, Texas Parks and Wildlife Department; MITCH LOCKWOOD, Big Game Warden, Texas Parks and Wildlife Department; DALE WATERS, Game Warden, Texas Parks and Wildlife Department; GARY COLLINS, Game Warden, Texas Parks and Wildlife Department; BRAD CHAPPELL, Investigator, Texas Parks and Wildlife Department; AMBER ANDEL, Deer Breeders Program, Texas Parks and Wildlife Department, Defendants – Appellees

SNIP...

FACTUAL AND PROCEDURAL BACKGROUND

From 2000 until 2010, the Andertons possessed a current permit issued by the Department that authorized them to breed deer. Their breeding ranch was located east of Dallas in Quinlan, Texas. In 2010, 2011, and 2012, the Andertons submitted applications for renewal of the permit. The Department denied those applications without explanation.

It has been alleged in this litigation that the Andertons’s permit was not renewed because they had illegally imported deer from another state. The Andertons had pled guilty, as shown in a federal court judgment of February 2, 2010, to the offense of conspiring to transport wildlife in interstate commerce in violation of Texas law. The Andertons were charged under the general federal conspiracy statute, 18 U.S.C. § 371. The substantive federal statutes setting forth the offense were 16 U.S.C. §§ 3372(a)(2)(A) and 3373(d)(2). Those are sections of what is called the Lacey Act. 16 U.S.C. §§ 3371-3378. The Texas laws they were charged in an information with violating were 31 Texas Administration Code Sections 65.609(b)(2), 65.611(h), and 65.611(i). The Department may refuse permit issuance or renewal to any person convicted of a violation of the Lacey Act. 31 TEX. ADMIN. CODE § 65.603(g)(2) (2010).

As mentioned above, the Andertons deer-breeding permit was not renewed in 2010. Such a permit is valid from the date of issuance until the immediately following July 1. 31 TEX. ADMIN. CODE 65.603(c). Apparently, then, as of July 1, 2010, four months after their February convictions, they no longer had a current permit to maintain breeder deer. On December 6, 2010,

Case: 14-10297 Document: 00512982550 Page: 2 Date Filed: 03/26/2015

No. 14-10297

and May 1, 2011, Department officials entered and shot breeder deer on the Andertons’ land. The Andertons allege the officials killed the deer in front of their family and several witnesses. In April 2013, the Andertons brought suit in the United States District Court for the Northern District of Texas against the Texas Animal Health Commission and the Department for violations of the Fourth, Fifth, and Fourteenth Amendments, and the Racketeer Influenced and Corrupt Organizations Act (“RICO”). They also sued several Department officials in their official and individual capacities under 42 U.S.C. § 1983 and RICO. Additionally, they brought claims for injunctive and declaratory relief against all parties. The district court dismissed the Andertons’ Section 1983 and RICO claims against the officials in their individual capacities under Federal Rule of Civil Procedure 12(b)(6), and dismissed the remaining claims under Rule 12(b)(1). The Andertons appealed.

DISCUSSION

SNIP...

The Andertons argue that the breeder-deer industry is not a closely regulated industry.

SNIP...

The Andertons argue the killing of the deer “shocks the conscience.” Even if there were a protected property interest in the deer – an issue we will analyze in the next section on procedural rights – Department officials were acting pursuant to state law, not violating it. The Andertons failed to allege facts that established the conduct of the officials was intended to injure them unjustifiably. The district court stated that though the allegations “may raise questions related to the most humane ways to kill deer, [they] do not plausibly establish that the officials’ conduct was so egregious, so outrageous, that it may fairly be said to shock the contemporary conscience.”

The Andertons have failed to demonstrate a violation of substantive due process under the Fourteenth Amendment.

SNIP...

Under Texas law, though, breeder deer belong to the state, not the permittee. See, e.g., TEX. PARKS & WILD. CODE §§ 1.011 (“All wild animals . . . inside the borders of this state are the property of the people of this state.”); 43.364 (“All breeder deer . . . are under the full force of the laws of [Texas] pertaining to deer . . . .”). While a permittee may have possession of the breeder deer, the deer are only “held under a permit[.]” Id. § 43.351. Nowhere do the statutes or regulations state that breeder deer become the property of a permit holder.4 Regardless, even if they did give ownership of breeder deer to permit holders, the Andertons were not permit holders when the deer were killed.

http://www.ca5.uscourts.gov/opinions%5Cunpub%5C14/14-10297.0.pdf

While a permittee may have possession of the breeder deer, the deer are only “held under a permit[.]” Id. § 43.351

http://www.statutes.legis.state.tx.us/Docs/PW/htm/PW.43.htm

S.B. No. 820

http://www.legis.state.tx.us/tlodocs/83R/billtext/html/SB00820F.HTM

http://openstates.org/tx/bills/83/SB820/

Texas Senate Bill

Relating to the management, breeding, and destruction of deer and to procedures regarding certain deer permits.

View latest bill text Session:83rd Legislature (2013)

http://openstates.org/tx/bills/83/SB820/

Deer Smugglers Go to Prison

A Texas man and a Minnesota man have been sent to prison for transporting wild deer across state lines. You may not have known that is illegal, but it is, and the Texas Parks and Wildlife Department takes it very seriously, as Jim Bell reports.

By: Jim Bell, December 29th, 2008 08:21 PM

00:00 00:00

Everyone knows it's legal to hunt wild deer in Texas under controlled and licensed conditions. It's also legal for people to get a license to breed deer on private property and use them for private hunts. What's not legal is for the deer breeders to bring deer in from other states. Greg Williford is in the Texas Parks and Wildlife Department's Special Operations Unit, and he says deer from other states could bring a very serious animal disease into the state. It's called Chronic Wasting Disease.

"Similar to Mad Cow Disease but it affects deer and elk. It's been found in Wisconsin and I believe Colorado had it in their elk population at one point.Î¾ It completely decimates the native deer population."

Deer hunting is a huge industry in Texas. Add up the money hunters spend year round on hunting rifles, hunting and camping clothing and equipment, deer leases, everything related to traveling to hunting areas, hotels, motels, campgrounds, and you're talking about hundreds of millions of dollars. A sick deer from another state could start an epizootic that could spread and kill thousands of deer, and do serious damage to the state's hunting economy.Î¾

What were those two men doing that got them in so much trouble?Î¾ Williford says it started when Robert Eichenour of Houston bought mature trophy bucks with big racks from a rogue breeder named Brian Becker in Minnesota.

"Mr. Eichenour was able to buy them from Mr. Becker in Minnesota for approximately 1500 dollars a buck.Î¾ Mr. Becker would bring the animals down, they'd be placed on Mr. Eichenour's ranch in Grimes County and then he would sell the hunt for this animal for anywhere between five and ten thousand dollars."Î¾

And that's per hunter. Over a four year period, Eichenour smuggled up to $300 thousand dollars worth of deer to his ranch for these hunts. The question arises: since he could breed deer legally as a licensed breeder, why did he need to import deer?Î¾ Williford says:

"It was more economically advantageous for him to import these deer than it was to try to raise these deer. It would take four to five years to raise a deer with antlers of this quality anyway."

Williford says the Texas breeder just didn't want to spend the time and money it takes to raise trophy deer to adulthood. Both men were caught after an undercover investigation by Parks and Wildlife Department Game Wardens, and both were charged with illegally transporting illegally captured animals across state lines, which is a federal crime. Last month, both pleaded guilty and both are headed for a federal prison. Williford says they can blame it on greed.Î¾

Jim Bell, KUHF, Houston Public Radio News.

http://www.houstonpublicmedia.org/news/1230585678/

Owner of Sandia Deer Breeding Facility Bonded Out of Jail

Posted: May 08, 2014 6:07 PM CDT

Updated: May 16, 2014 6:54 PM CDT

ANDIA (Kiii News) - Fred Rich, the man arrested Wednesday for allowing someone to enter a deer breeding facility and kill a 22-point buck while it was still inside a pen, bonded out of jail on Thursday.

Rich is said to be the owner of the breeding facility at the Lonesome Bull Ranch near Sandia, Texas. That facility is currently being investigated by Texas Parks and Wildlife, and that's how the incident was discovered.

Parks and Wildlife officers are also testing whitetail dear from that ranch for an infectious disease. More than 200 of the deer had to be euthanized to allow for that testing, and the results are expected back in a few weeks. As for Rich, if convicted in aiding the slaughtering of that deer, he could face up to six months in jail.

It's not his first run-in with the law. Rich received time in prison for federal charges that he scammed insurance companies out of thousands of dollars in fraudulent mold claims.

Prosecutors said Rich, who owned a company called Mold Inc., and two other men pled guilty to suing a technique called "cooking houses." It involved wetting down the inside of a home, shutting doors and windows, and then cranking up the heat to create mold.

The alleged incidents happened in Corpus Christi and in Portland between 2000-2002.

http://www.kiiitv.com/story/25469665/owner-of-sandia-deer-breeding-facility-bonded-out-of-jail

Geneticist Fined in Texas Deer Semen Case

An Illinois geneticist must pay $30,000 and serve three years of probation for illegally obtaining valuable semen from whitetail deer in East Texas. A federal judge in Tyler sentenced 55-year-old Raymond Favero of Braidwood, Ill. Favero in...

http://article.wn.com/view/2012/12/20/Geneticist_Fined_in_Texas_Deer_Semen_Case/

SHOCKING ONES CONSCIENCE VIDEO

*** Danger of Canned Hunting Indiana Wildlife VIDEO ***

http://vimeo.com/5680646

2021 TEXAS CHRONIC WASTING DISEASE CWD TSE PRION

Media Contacts: TAHC Communications, public_info@tahc.texas.gov, 512.719.0750

TPWD Press Office, news@tpwd.texas.gov, 512.389.8030

For Immediate Release August 27, 2021

Chronic Wasting Disease Discovered at a Deer Breeding Facility in Duval County

AUSTIN, TX – Chronic Wasting Disease (CWD) has been discovered in a deer breeding facility in Duval County, marking the first positive detection of the disease in the county.

The tissue samples were submitted by the deer breeding facility as part of required CWD surveillance programs. The samples indicated the presence of CWD during testing at the Texas A&M Veterinary Medical Diagnostic Laboratory (TVMDL) in College Station on August 18. The National Veterinary Services Laboratory in Ames, Iowa confirmed CWD in those tissue samples on August 25.

Officials have taken immediate action to secure all deer at the facility and plan to conduct additional investigations for CWD. In addition, other breeding facilities that received deer from this facility or shipped deer to this facility during the last five years are under movement restrictions and cannot move or release deer at this time.

Texas Parks and Wildlife Department (TPWD) and Texas Animal Health Commission (TAHC) are working together to determine the extent of the disease within the facility and evaluate risks to Texas’ free ranging deer populations. Quick detection of CWD can help mitigate the disease’s spread.

“Early detection and containment remain critical strategies in our efforts to help mitigate the risk of further spread of this disease,” said Carter Smith, TPWD Executive Director. “There is too much at stake across Texas to do otherwise.”

Animal health and wildlife officials will conduct an epidemiological investigation in an effort to determine the source and potential dispersal of the disease from this facility, but the nature of CWD will likely prevent definitive findings.

“The incubation period of CWD can span years creating disease detection and management challenges,” said Dr. Andy Schwartz, TAHC State Veterinarian. “Response staff are diligently working to address each herd affected by this new detection to manage further spread.”

CWD was first recognized in 1967 in captive mule deer in Colorado. CWD has also been documented in captive and/or free-ranging deer in 26 states and 3 Canadian provinces.

Media Contacts: TAHC Communications, public_info@tahc.texas.gov, 512.719.0750 TPWD Press Office, news@tpwd.texas.gov, 512.389.8030

CWD is a fatal neurological disease found in certain cervids, including deer, elk, moose and other members of the deer family. CWD is a slow and progressive disease. Due to a long incubation, cervids infected with CWD may not produce any visible signs for a number of years after becoming infected. As the disease progresses, animals with CWD show changes in behavior and appearance. Clinical signs may include progressive weight loss, stumbling or tremors with a lack of coordination, excessive thirst, salivation or urination, loss of appetite, teeth grinding, abnormal head posture, and/or drooping ears.

To date there is no evidence that CWD poses a risk to humans or non-cervids. However, as a precaution, the U.S. Centers for Disease Control and the World Health Organization recommend not to consume meat from infected animals.

For more information about CWD, visit the TPWD web site or the TAHC web site. ###

The Texas Animal Health Commission (TAHC) was established in 1893 as the Livestock Sanitary Commission and charged with protecting the state’s domestic animals “from all contagious or infectious diseases of a malignant character.” TAHC remains true to this charge while evolving with the times to protect the health and marketability of all Texas livestock and poultry. Learn more about the TAHC by visiting www.tahc.texas.gov.

https://www.tahc.texas.gov/news/2021/2021-08_27_CWD.pdf

TAHC 409TH COMMISSION MEETING JUNE 29, 2021 CWD CALL FOR SPECIAL MEETING DENIED, DELAYED TO NEXT MEETING

TAHC 409TH COMMISSION MEETING CWD TSE PRION JUNE 29, 2021

TAHC 409TH COMMISSION MEETING JUNE 29, 2021

MEETING starts out by a Commissioner questioning why the first presenter did not address CWD, and speaker said that Dr. Susan Rollo would speak on CWD, and that a nice lady TAHC Commissioner (no named mentioned) that was very concerned about CWD, and she called for a special meeting on CWD, but she was shot down by another lady that said that would not be possible, that cwd would have to be address at next meeting. passing the buck again...so sad. here's what i wrote down.

CWD DR. SUSAN ROLLO MINUTE MARK 59:20

MARCH 23, 2021

5 CWD POSTIVE AT 2 DIFFERENT FACLITIES OWNED BY SAME OWNER UVALDE COUNTY

TO DATE, 7 POSITIVE CWD AT UVALDE FACILITY 1

AND

15 POSITIVE CWD AT FACILITY 2, WHICH IS A 4TH YEAR CERTIFIED HERD. WE ARE IN THE PROCESS OF DEPOPULATION OF THIS HERD.

1 DOE WAS POSITIVE HUNT COUNTY

SEE FULL MEETING OVER 4 HOURS, and it's very discouraging;

https://www.youtube.com/watch?v=w41tLvOkT8A

Texas Kimble County Farm Chronic Wasting Disease CWD TSE Prion Approximate Herd Prevalence 12%

SUMMARY MINUTES OF THE 407th COMMISSION MEETING Texas Animal Health Commission

September 22, 2020

Chronic Wasting Disease (CWD):

A new CWD positive breeding herd was disclosed in February 2020 in Kimble County. This herd depopulation was completed in July 2020. Including the two index positive deer, an additional eight more positive deer were disclosed (approximate herd prevalence 12%). Since July 2015 and prior to this discovery, five positive captive breeder herds have been disclosed and four of those are in Medina County. One herd in Lavaca and three herds in Medina County were depopulated leaving one large herd in Medina County that is managed on a herd plan. A new zone was established in Val Verde County in December 2019 as a result of a positive free-ranging White-tailed Deer (WTD). A second positive WTD was also disclosed in February 2020 in the same area.

https://www.tahc.texas.gov/agency/meetings/minutes/Minutes_CommMtg_2020-09-22.pdf

https://tpwd.texas.gov/newsmedia/releases/?req=20210514a

https://tpwd.texas.gov/newsmedia/releases/?req=20210331b

MONDAY, JULY 26, 2021

TAHC 409TH COMMISSION MEETING JUNE 29, 2021 CWD CALL FOR SPECIAL MEETING DENIED, DELAYED TO NEXT MEETING

deeply disturbing, discouraging, and very concerning...terry

https://chronic-wasting-disease.blogspot.com/2021/07/tahc-409th-commission-meeting-june-29.html

TITLE 31. NATURAL RESOURCES AND CONSERVATION

PART 2. TEXAS PARKS AND WILDLIFE DEPARTMENT

CHAPTER 65. WILDLIFE

SUBCHAPTER B. DISEASE DETECTION AND RESPONSE

DIVISION 1. CHRONIC WASTING DISEASE (CWD)

31 TAC §65.100

The Texas Parks and Wildlife Commission adopts new §65.100, concerning Emergency Provisions, on an emergency basis. The emergency action is necessary because of the recent discovery of chronic wasting disease (CWD) in six deer breeding facilities ("positive facilities"), five of which have extensive direct or indirect epidemiological connectivity with each other and/or other deer breeding facilities and locations where breeder deer have been released, introducing a significant possibility that free-ranging deer populations in the state have been exposed to CWD. CWD is a fatal neurodegenerative disorder that affects cervid species such as white-tailed deer, mule deer, elk, red deer, sika, and others (susceptible species). The department, along with the Texas Animal Health Commission (TAHC), has been engaged in an ongoing battle with CWD in Texas since 2002. White-tailed deer and mule deer are species authorized to be regulated by the department under the Parks and Wildlife Code. TAHC is the state agency charged with disease management in livestock and exotic livestock, including exotic livestock known to be susceptible to CWD. The recent detections of CWD in breeding facilities create an unprecedented situation because they are at a scale that is orders of magnitude greater than earlier instances of detection encountered by the department.

When CWD is detected in a facility, that facility and any directly connected facility are immediately prohibited by current rule from receiving or transferring deer and the department and TAHC staff immediately begin epidemiological investigations to determine the extent and significance of possible disease transmission. In this case, department records indicate that within the last five years, the positive facilities referenced earlier transferred a total of 1,709 deer to 138 deer breeding facilities and 103 release sites located in a total of 56 counties. These breeding facilities and release sites are therefore directly connected to at least one of the positive facilities and by current rule were designated as having not movement qualified status (prohibited from receiving or transferring deer). As a result of the ongoing epidemiological investigation and pursuant to existing regulations, 66 of the 138 directly connected breeding facilities have regained movement qualified status if otherwise eligible, leaving 72 facilities of epidemiological concern. An additional 214 deer breeding facilities received deer from one or more of those 72 directly connected breeding facilities; these facilities are indirectly connected to the positive facilities and are of epidemiological concern because it is possible that within the last five years any or all of them could have received CWD-infected deer. The five-year window is important because (based on the literature) it encompasses the time period from possible exposure to CWD, through the incubation period, to the time at which the disease can be transmitted to another animal or the environment. Current rules do not address disease response with respect to indirectly connected facilities. The emergency rules will address that situation by imposing requirements for disease testing and movement of breeder deer to and from indirectly connected facilities. In addition, the rules will require ante-mortem testing of all age eligible deer prior to being transferred to a release site. The department and TAHC will continue to conduct epidemiological investigations and the department will undertake rulemaking through the normal administrative process.

CWD is classified as a TSE (transmissible spongiform encephalopathy), a family of diseases that includes scrapie (found in sheep) and bovine spongiform encephalopathy (BSE, found in cattle and commonly known as Mad Cow Disease). CWD is transmitted both directly (through deer-to-deer contact) and indirectly (through environmental contamination). Much remains unknown about CWD. The peculiarities of its transmission (how it is passed from animal to animal), infection rate (the frequency of occurrence through time or other comparative standard), incubation period (the time from exposure to clinical manifestation), and potential for transmission to other species are still being investigated; however, a high prevalence of the disease in free-ranging populations is correlated with significant deer population declines as well as expected negative impacts and influences on landowners, hunting, and hunters. The implications of CWD for Texas and its multi-billion-dollar ranching, hunting, and wildlife management economies are significant.

The department has engaged in several rulemakings over the years to address the threat posed by CWD. In 2013, based on recommendations from the CWD Task Force, the department adopted rules (37 TexReg 10231) to implement a CWD containment strategy in response to the first detection of CWD in Texas. In 2015, the department discovered CWD in a deer breeding facility in Medina County and adopted emergency rules (40 TexReg 5566) to respond immediately to the threat, followed by interim rules (41 TexReg 815) intended to function through the 2015-2016 hunting season. Working closely with TAHC and with the assistance of the Center for Public Policy Dispute Resolution of the University of Texas School of Law, the department developed and adopted comprehensive CWD management rules in 2016 (41 TexReg 5726). The department has since engaged in periodic rulemaking (both emergency and via the normal rulemaking process) to create containment and surveillance zones in response to CWD detections in both free-ranging and captive deer in various parts of the state.

The emergency rule sets forth specific testing requirements for certain deer breeders that would have to be satisfied in order for the department to restore movement qualified status (authorization to resume transfers of deer into and out of a breeding facility). The emergency action is necessary because the risk to the multi-billion-dollar deer hunting and deer breeding industries represented by even one infected animal among a wild population is considerable. The department strongly believes that vigilance is crucial to minimizing the severity of biological and economic impacts that could result from the current situation, and that a more rigorous testing protocol at certain deer breeder facilities, pending resolution of the epidemiological uncertainty surrounding the spread of CWD from directly connected facilities, is a wise and responsible course of action.

The rules are adopted on an emergency basis under Parks and Wildlife Code, Chapter 43, Subchapter L, which authorizes the Parks and Wildlife Commission to establish regulations governing the possession of white-tailed and mule deer under a deer breeders permit; Parks and Wildlife Code, §12.027, which authorizes the Texas Parks and Wildlife Commission and the department's executive director to adopt emergency rules if there is an immediate danger to a species authorized to be regulated by the department, and under Government Code, §2001.034, which authorizes a state agency to adopt such emergency rules without prior notice or hearing.

§65.100.Emergency Provisions.

(a) Effectiveness. To the extent that any provision of this section conflicts with any provision of this division, the provisions of this section prevail.

(b) Definitions. The following words and terms shall have the following meanings, unless the context clearly indicates otherwise. All other words and terms used in this section shall have the meanings assigned by the Parks and Wildlife Code and §65.90 of this title (relating to Definitions).

(1) Exposed deer--A deer that meets any of the following criteria:

(A) the deer is or has been in a breeding facility where a CWD-positive deer has been kept following the date the facility was first exposed to CWD (if known);

(B) the deer is or has been in a breeding facility within the five-year period preceding the death date of any CWD-positive deer that was in the facility (or the date of a positive ante-mortem test result);

(C) the deer is in a breeding facility on or after the date that the facility received a deer under the circumstances described in subparagraph (A) or (B) of this paragraph; or

(D) the deer entered a Tier 1 facility from a trace-out breeding facility.

(2) Exposure--The period of time that has elapsed following the introduction of an exposed deer to a breeding facility.

(3) "Insufficient follicles"--A test result indicating that a tonsil or rectal biopsy sample contained an insufficient number of lymphoid follicles to produce a valid test result.

(4) Last known exposure--

(A) The later of:

(i) the last date a deer in a breeding facility was exposed to trace deer because of the death or transfer of that trace deer; or

(ii) the last date a deer entered a trace-out breeding facility after that facility received a trace deer; or

(B) the last date that a deer entered a Tier 1 facility after an exposed deer entered a Tier 1 facility.

(5) Test-eligible deer--A deer at least nine months of age.

(6) Tier 1 facility -- A breeding facility that has received an exposed deer from a trace-out breeding facility.

(7) Trace deer--A deer that the department has determined had been in a CWD-positive deer breeding facility on or after the date the facility was first exposed to CWD, if known; otherwise, within the previous five years from the reported mortality date of the CWD-positive deer, or the date of the ante-mortem test result.

(8) Trace-out breeding facility--A breeding facility that has received trace deer from a CWD-positive deer breeding facility.

(1) Deer required to be reported to the department under §65.605 of this title (relating to Holding Facility Standards and Care of Deer) are considered to be mortalities for the purposes of this subchapter until lawfully recaptured. A deer that is not recaptured will be treated as a mortality that occurred within the facility from which the escape is required to be reported.

(2) Deer that according to department records should be present in a breeding facility but cannot be accounted for to the satisfaction of the department are considered to be mortalities for the purposes of this subchapter.

(3) For facilities required to test ten or more deer, the department will not accept inconclusive ante-mortem test results, including but not limited to "insufficient follicles," for more than 10 percent of the total number of deer tested. For facilities required to test less than ten deer, inconclusive ante-mortem test results, including but not limited to "insufficient follicles," will not be accepted.

(d) Category A trace-out breeding facility.

(1) A Category A facility is a trace-out breeding facility:

(A) in which all trace deer are alive in the facility; or

(B) for which post-mortem test results of "not detected" have been returned for trace deer that have died and all other trace deer are alive and present in the facility.

(2) Upon notification by the department of Category A status, a permittee shall:

(A) within seven days euthanize all trace deer in the breeding facility and submit test samples for each of those deer for post-mortem testing within one business day, unless ante-mortem testing has been authorized under a herd plan approved by the department and TAHC, for a facility that:

(i) contains no deer that had been in a CWD-positive facility within the five-year period preceding notification of Category A status; or

(ii) had not transferred any deer to any location since receiving any deer from a CWD-positive facility; and

(B) inspect the facility daily for mortalities;

(D) immediately collect test samples from all test-eligible mortalities that occur within the facility and submit the samples for post-mortem testing within one business day of collection.

(5) The department will not restore MQ status unless CWD "not detected" test results are obtained for all required sample submissions and the permittee has complied with all applicable requirements of this subsection and this division.

(e) Category B trace-out breeding facility.

(1) A Category B facility is a trace-out breeding facility in which less than 100% of the trace deer that department records indicate were received by the facility are for whatever reason (including but not limited to transfer, release, or escape) available for testing.

(2) Upon notification by the department of Category B status, a permittee shall:

(A) within seven days euthanize all trace deer in the breeding facility and submit test samples for each of those deer for post-mortem testing within one business day;

(B) inspect the facility daily for mortalities;

(D) immediately collect test samples from all test-eligible mortalities that occur within the facility and submit the samples for post-mortem testing within one business day of collection; and

(E) conduct ante-mortem testing of all test-eligible deer in the facility as specified in the following:

(i) for a facility for which the date of last known exposure is within the immediately preceding 18 months:

(I) submit rectal or tonsil biopsy samples collected on or after April 1, 2021; and

(II) submit tonsil biopsy samples collected no earlier than 24 months from the date of last known exposure;

(ii) for a facility for which the date of last known exposure is not within the immediately preceding 18 months and not at a time prior to the immediately preceding 36 months: collect and submit tonsil biopsy samples no earlier than 24 months from the date of last known exposure; and

(iii) for a facility for which the date of last known exposure occurred at a time after the immediately preceding 36 months: collect and submit rectal or tonsil biopsy samples collected no earlier than 36 months from the date of last known exposure.

(F) Samples required by subsection (e)(2)(E) of this section shall submitted no later than 45 days after the applicable last known exposure period as determined by the department.

(3) The department will not restore MQ status unless CWD "not detected" test results are obtained for all required sample submissions and the permittee has complied with all applicable requirements of this subsection and this division.

(f) Tier 1 facility.

(1) Upon notification by the department of Tier 1 status, a facility is automatically NMQ and the permittee shall:

(A) inspect the facility daily for mortalities;

(B) immediately report all test-eligible deer mortalities that occur within the facility; and

(C) immediately collect test samples from all test-eligible deer mortalities that occur within the facility and submit for post-mortem testing within one business day of collection.

(2) The department will not restore MQ status unless the permittee has complied with all applicable requirements of this subsection and this division, and:

(A) post-mortem results of "not detected" have been submitted for every exposed deer received from a trace facility;

(B) the department has restored MQ status to all trace facilities from which deer were received; or

(g) Release of breeder deer.

(1) No person may transfer a breeder deer to a release facility or cause or allow a breeder deer to be transferred to a release facility unless an ante-mortem test on rectal or tonsil tissue collected from the deer within the six months immediately preceding the release has been returned with test results of "not detected."

(2) A facility from which deer are transferred in violation of paragraph (1) of this subsection becomes automatically NMQ and any further transfers are prohibited until the permittee has conducted ante-mortem testing as specified in writing by the department.

(h) Violations and Penalties. In addition to any other conduct punishable as a violation of this subchapter or the Parks and Wildlife Code, it is an offense for any person to fail to abide by any provision of this section, a permit provision, or a herd plan.

The agency certifies that legal counsel has reviewed the emergency adoption and found it to be within the state agency's legal authority to adopt.

Filed with the Office of the Secretary of State on June 22, 2021.

TRD-202102396

James Murphy

General Counsel

Texas Parks and Wildlife Department

Effective date: June 22, 2021

Expiration date: October 19, 2021

For further information, please call: (512) 389-4775

https://www.sos.texas.gov/texreg/archive/July22021/Emergency%20Rules/31.NATURAL%20RESOURCES%20AND%20CONSERVATION.html

TEXAS CHRONIC WASTIND DISEASE CWD TSE PRION CASES JUMPS TO 228 CONFIRMED

https://tpwd.texas.gov/newsmedia/releases/?req=20210622a

Texas Emergency Order Issued to Restrict Movement of Deer from Breeding Facilities Where CWD has Been Detected

PRICE OF CWD TSE PRION TEXAS HOLDEM POKER GOES UP $$$

Emergency Order Issued to Restrict Movement of Deer from Breeding Facilities Where CWD has Been Detected

June 22, 2021 Media Contact: TPWD News, Business Hours, 512-389-8030 News Image Share on Facebook Share Release URL

AUSTIN – Texas Parks and Wildlife Department (TPWD) has issued an “emergency order” to impose additional movement and testing restrictions on deer breeding facilities that are affiliated with six deer breeding facilities where Chronic Wasting Disease (CWD) has been positively detected. Existing rules already restrict the movement of deer from 264 sites in 95 counties that are directly linked to these CWD-positive facilities, but further measures are necessary given the gravity of this situation.

TPWD and Texas Animal Health Commission (TAHC) are addressing risks and improving management strategies to protect big game resources from CWD in captive or free-ranging cervid populations. Both agencies recognize the need for full cooperation and partnership among government agencies, deer breeders, private landowners, hunters, conservation organizations and the general public in managing CWD in Texas.

“This is a terribly unfortunate development that we are committed to addressing as proactively, comprehensively, and expeditiously as possible,” said Carter Smith, TPWD Executive Director. “The health of our state’s free-ranging and captive deer herds, as well as affiliated hunting, wildlife, and rural based economies, are vitally important to Texas hunters, communities, and landowners. As such, our primary objectives are to enhance testing at sites that received deer from affected facilities and avoid the unintentional release of CWD-positive deer. Along with our partners at TAHC, we will continue to exercise great diligence and urgency with this ongoing investigation.”

Officials have taken action to secure all cervids at the CWD-positive facilities with plans to conduct additional investigations for CWD. In addition, those breeding facilities that received deer or shipped deer to those facilities during the last five years are under movement restrictions and cannot move or release cervids until cleared by a herd plan. The additional measures included in this emergency order include enhanced testing requirements for facilities with close epidemiological ties to the CWD-positive facilities and antemortem testing of deer from all movement qualified deer breeding facilities prior to transfer to a release site. These requirements are necessary to further minimize risk of CWD spreading into Texas’ free-ranging white-tailed deer herd, and to protect the captive deer breeding industry.

“The TAHC is committed to working with TPWD and affected stakeholders and landowners to address this latest development in Texas’ CWD history,” said Dr. Andy Schwartz, TAHC Executive Director and State Veterinarian. “The TAHC will continue to use its veterinary and epidemiological expertise to facilitate and contribute to the state’s CWD herd management and surveillance strategies.”

“The Texas Parks and Wildlife Commission and TPWD are deeply concerned about the gravity and the urgency of the CWD challenges now confronting us,” said Arch H. “Beaver” Aplin, III Chairman TPW Commission. “Please also know that the engagement and input from everyone interested in deer management will be important as we work together along with TAHC to try and arrest the spread of this insidious disease.”

“I am proud of the partnership between TAHC and TPWD and the dedication of the two agencies to address Chronic Wasting Disease in this state,” said Coleman Locke, TAHC Chairman. “Because of this collaboration, Texas has led the nation in CWD management techniques and will continue to improve the overall understanding of the disease.”

As the state veterinary diagnostic laboratory, the Texas A&M Veterinary Medical Diagnostic Laboratory (TVMDL), uses surveillance testing to help wildlife producers and wildlife/animal health agencies to ensure the health of species susceptible to CWD. With the expected increase in CWD testing, TVMDL, a state agency within the Texas A&M System, has committed additional resources to ramp up testing capacity.

“Working with TPWD, the Texas A&M System will use all of its resources to perform these tests quickly and efficiently,” said Texas A&M University System Chancellor John Sharp.

CWD was first recognized in the United States in 1967 and has since been documented in captive and/or free-ranging deer in 26 states and three Canadian provinces.

CWD is a fatal neurological disease found in certain cervids, including deer, elk, moose and other members of the deer family. CWD is a slow and progressive disease. Due to a long incubation, cervids infected with CWD may not produce any visible signs for a number of years after becoming infected. As the disease progresses, animals with CWD show changes in behavior and appearance. Clinical signs may include, progressive weight loss, stumbling or tremors with a lack of coordination, excessive thirst, salivation or urination, loss of appetite, teeth grinding, abnormal head posture, and/or drooping ears.

For more information about CWD, visit the TPWD Chronic Wasting Disease page or the TAHC web site.

A full list of TVMDL’s test offering are available on the TVMDL website and laboratory experts are available to answer any questions by calling 979-845-3414.

Emergency Order Issued to Restrict Movement of Deer from Breeding Facilities Where CWD has Been Detected

June 22, 2021

https://tpwd.texas.gov/newsmedia/releases/?req=20210622a

Official Update from the TPWD Wildlife Permit Office

Dear Texas Deer Breeder,

Emergency rules have been adopted in response to the discovery of CWD positive deer in 6 facilities since March 23, 2021.

As of June 21, 2021, the following regulations have been enacted and apply to all permitted deer breeding facilities:

All deer of eligible age (9 months and older) must have a “Not Detected” rectal or tonsil biopsy test result within the six month period prior to transfer to a release site.

Transferring deer to a release site without a valid ante mortem test as required is a violation that will result in immediate loss of Movement Qualified status and subject to the CWD testing requirements required by Parks and Wildlife Code §65.94(f) and may result in subsequent permit denial. All escaped deer required to be reported to TPWD under §65.605 of this title (relating to Holding Facility Standards and Care of Deer) will be considered mortalities for the purposes of this subchapter until lawfully recaptured. A deer that is not recaptured will be treated as a mortality that occurred within the facility from which the escape is required to be reported. Deer that according to department records should be present in a breeding facility but cannot be accounted for to the satisfaction of TPWD will be considered mortalities.

Additional information will follow for facilities who have been identified as Trace or Tier 1 facilities.

If you have any questions please contact the Wildlife Permitting Office at 512-389-4585 or deer.breeder@tpwd.texas.gov.

=====end=====

COMPREHENSIVE CWD MANAGEMENT RULES ADOPTION PREAMBLE

1. Introduction.

The Texas Parks and Wildlife Commission (Commission) in a duly noticed meeting on June 20, 2016 adopted the repeal of §§65.90-65.94 and new §§65.90-65.99 concerning Chronic Wasting Disease - Movement of Deer. New §§65.90-65.92 and 65.94-65.98 are adopted with changes to the proposed text as published in the April 22, 2016, issue of the Texas Register (41 TexReg 2853). The repeals, new §65.93, and new §65.99 are adopted without changes and will not be republished.

Change to Definitions

The change to §65.90, concerning Definitions, adds a definition for “Interim Breeder Rules.” The rules as adopted, include provisions predicated upon compliance with previous rules. Therefore, a definition of “Interim Breeder Rules” was added to provide a shorthand reference for those rules.

Changes to General Provisions

The change to §65.91, concerning General Provisions, alters subsection (a). As proposed, the subsection provided that to the extent any provision of the proposed new rules conflicted with any other provision of Chapter 65, the new rules would control; however, Chapter 65, Subchapter B, Division 1 contains provisions regarding Chronic Wasting Disease (CWD) management zones that are intended to function on their own terms. Therefore, it is necessary to clarify that fact.

The change to §65.91 also adds new subsection (d) to clarify that a deer breeding facility is prohibited from moving deer out of the breeding facility if such movement is prohibited under a hold order or quarantine imposed on the breeding facility by the Texas Animal Health Commission (TAHC). Under the rules as proposed, and as adopted, the lawful movement of breeder deer is predicated on a facility’s designation as Movement Qualified (MQ). Although the provisions of §65.99 as adopted, concerning Violations and Penalties, provide that a person who possesses or receives white-tailed deer or mule deer pursuant to a Triple T permit, DMP or

a deer breeder permit is subject to the provisions of TAHC regulations regarding Chronic Wasting Disease, subsection (d) was clarified to state that if a facility is prohibited from moving deer under a hold order or quarantine issued by TAHC, movement of deer under those circumstances is prohibited.

In addition, a change was made to §65.91(e) (which was §65.91(d) as proposed) to modify the cross-reference to the subsection regarding receipt of deer by a release site. The rules as adopted adjust the time period for release site testing. As a result, it is more appropriate to reference the entirety of §65.95(c) regarding release sites, rather than the more specific §65.95(c)(1)(D).

Changes to CWD Testing

The change to §65.92, concerning CWD Testing, clarifies who may collect the tissue upon which ante-mortem tests are to be conducted and provides additional detail to ensure that a valid sample is collected. As proposed, §65.92(b) required ante-mortem test samples be collected “by or under the supervision of a qualified licensed veterinarian.” However, to ensure compliance with statutory requirements regarding the practice of veterinary medicine, as well as regulatory requirements of TAHC and the Texas Board of Veterinary Medical Examiners regarding the collection of ante-mortem samples, the change to §65.92(b) provides that antemortem samples must be collected by a “licensed veterinarian authorized pursuant to statutes and regulations governing the practice of veterinary medicine in Texas and regulations of the TAHC.” In addition, to ensure that samples are sufficient to accommodate ante-mortem testing, §95.92(b) was modified to require that at least six lymphoid follicles be collected.

The change to §65.92(b) also eliminates the 16-month residency requirement for antemortem testing of breeder deer imposed by proposed subsection (b)(2). The intent of proposed subsection (b)(2) was to ensure that animals subjected to ante-mortem testing had been in a facility long enough to have contracted CWD if it were present. However, while a deer with a residency in a facility of less than 16 months may not have had sufficient incubation time to detect CWD if it was contracted in that facility, a test of that deer would provide information about any previous facility in which the deer was held. Therefore, the residency requirement was eliminated.

snip...see full text;

https://tpwd.texas.gov/huntwild/wild/diseases/cwd/docs/Comprehensive_CWD_Management_Rules.pdf

TEXAS CWD TSE Prion Urgent Letter to Honorable Arch H. Aplin, III from Officials and Land Owners

''the high rate of “lost” deer, and other inadequacies and loopholes in the current rules have put our state’s susceptible cervid herds at significant risk''

June 14, 2021

The Honorable Arch H. Aplin, III

Chairman, Texas Parks and Wildlife Commission

4200 Smith School Road

Austin, Texas 78744

Dear Chairman Aplin,

In light of the recent discovery of CWD in captive deer herds, the undersigned encourage the Commission to take swift and decisive action to protect our captive and wild deer herds, even up to an immediate partial or complete shut-down of deer movement. It is imperative that the response be focused not only on uncovering the sources of the infections, but also in promptly conducting all necessary trace-outs to determine the extent of disease spread.

We appreciate and value the efforts of the Commission and the Department staff to rapidly and effectively deal with this CWD outbreak, but as you are aware, and as staff at the Texas Parks and Wildlife Department (Department) have stated, the modeling used in establishing the current testing requirements has proven insufficient to detect CWD at any reasonable probability and degree of confidence. It is likely that CWD was present in the Uvalde County index facility for a year or more prior to detection, and over a hundred deer were transported to other breeders and release sites in the interim. Additionally, the combined effects of insufficient reporting, testing, and other non-compliance issues with current rules, the high rate of “lost” deer, and other inadequacies and loopholes in the current rules have put our state’s susceptible cervid herds at significant risk.

By the rights and privileges conveyed in the permitting process, deer breeders have accepted and taken calculated risks, for which they are ultimately responsible. While the Department has operated in good faith, it is also the Department’s responsibility to take any and all necessary actions required by their mission statement. The time has come for deer breeders and the Department to accept the necessity that additional steps must be taken to protect this incredibly valuable resource for the public good.

The undersigned strongly urge that the Commission promote enforcement of existing rules and regulations, including revocation, suspension, or non-renewal of non-compliant deer breeders, pursuant to Sunset recommendations. It is also imperative that the Commission promptly engage all appropriate stakeholders (CWD Taskforce, White-tailed Deer Advisory Committee, and Deer Breeder User Group) to review and bolster existing rules that have clearly proven insufficient to identify and contain this disease. In addition, we urge the Department to work closely with Texas Animal Health Commission (TAHC) and other regulatory agencies in expediting the trace-out process, strengthening the oversight of carcass disposal and transport, as well as strengthening the rules and testing related to exotics as a highest priority.

In closing, we express our strong support for tightening any loopholes that exist in current rules associated with CWD detection and containment. We also ask that the Department revisit all assessments made for probability of detection and correct deficits that might currently exist. It is imperative that our response to CWD in Texas move from a reactive to a proactive posture that more effectively protects this precious resource.

Again, we whole-heartedly support and value the Commission and the Department staff as you rapidly and effectively deal with this CWD outbreak, and we stand ready to assist and support you in any manner necessary.

Respectfully,

Terry Anderson – Nacogdoches County Landowner

Ernest Angelo Jr. - Former TPWD Commissioner

John Barrett – Mason County Landowner

Giovana L. Benitez, South Texans’ Property Rights Association Director, Hidalgo

County Landowner

George Bristol – Texas Foundation for Conservation

Dr. Fred C. Bryant – Texas Foundation for Conservation; Past President, Texas Chapter of The

Wildlife Society; Former Board Member, Rocky Mountain Elk Foundation; Professional

Member, Boone & Crockett Club

Rene Barrientos – South Texas Landowner

Emry Birdwell - Partner, Birdwell & Clark Ranch, Lone Star Land Steward Award Recipient

Rory Burroughs – Fisher County Landowner

Gus T. Canales – South Texas Landowner

Linda Campbell – Certified Wildlife Biologist; Past President, Texas Chapter of The Wildlife

Society

Dr. Jim Cathey – Past President – Texas Chapter of The Wildlife Society

Ken Cearley – Certified Wildlife Biologist, Cornerstone Ranching Solutions LLC

Michael J. Cherry - Wildlife Biologist

Deborah Clark - Partner, Birdwell & Clark Ranch, Lone Star Land Steward Award Recipient

Charles A. DeYoung - Ph D, Wildlife Biologist

Dr. Randy DeYoung - PhD, Fellow, The Wildlife Society

Don Dietz – Certified Wildlife Biologist

Cary Dietzmann – Washington County Landowner

Alice East – South Texans’ Property Rights Association, South Texas Landowner

Dr. Bill Eikenhorst – Veterinarian, Washington County Landowner

Jay C. Evans –Texas Landowner

Richard Guerra - Starr County landowner

Henry Hamman, South Texas Landowner

Trey Henderson – Angelina County Landowner

Dr. David G. Hewitt; Past President of the Texas Chapter of The Wildlife Society; Professional

Member, Boone and Crockett Club

Dr. Clayton D. Hilton – Veterinarian; Professional Member, Boone and Crockett Club

Gail & Bruce Hoffman - Jim Wells County Landowners

Anson B. Howard, Dimmit, Tom Green, and Coleman County Landowner

A.C. “Dick” Jones, IV - Jim Hogg County Landowner

W.W “Whit” Jones III - Jim Hogg County Landowner

David Kelly – Brooks County Ranch Manager, Leopold Award Recipient

Tio Kleberg – South Texas Landowner

Whitney Marion Klenzendorf – Frio County Landowner

Dr. Wallace Klussmann – Retired Head, Department of Wildlife and Fisheries Sciences, Texas

A&M University; Past President, Texas Chapter of The Wildlife Society; Founder, Texas

Youth Hunting Program

Jim Kolkhorst – Conservationist, Washington and Freestone County Landowner

Keith Lake – Wildlife Biologist

David K. Langford – Kendall County Landowner; Retired CEO, Texas Wildlife Association;

Lone Star Land Steward Award Recipient, Emeritus Member, Boone & Crockett Club

Berdon Lawrence – South Texans’ Property Rights Director, South Texas Landowner

Ken Leonard – South Texas Landowner

Roy Leslie – Low fence, no lease Kendall County Landowner; Lone Star Land Steward Award

Recipient

Dr. Roel Lopez – Past President – Texas Chapter of The Wildlife Society, Texas Foundation for

Conservation, Professional Member – Boone & Crockett Club

Chancey Lewis - Wildlife Biologist, Milam County Landowner

Steve C. Lewis – Kendall & Medina Landowner; Past President, Texas Wildlife Association

Ben Masters - Conservation Filmmaker, Writer

T. Nyle Maxwell – McCulloch and Hudspeth County Landowner

Coley Means – Culberson & Jeff Davis County Landowner

Jon Means – Culberson & Jeff Davis County Landowner; Past President, Texas & Southwestern

Cattle Raisers Association

Dr. Dan McBride – Veterinarian; Regular Member, Boone and Crockett Club, Burnet and

Hudspeth County Landowner

Robert F. McFarlane M.D. – Anderson County Landowner, Leopold Conservation Award

Recipient

Dr. James E. Miller - Past-President, The Wildlife Society; Honorary Member of The Wildlife

Society; Aldo Leopold Memorial Award Recipient

Brian Murphy – Certified Wildlife Biologist; Former CEO, Quality Deer Management

Association

Steve Nelle – Consulting Biologist

Eric Opiela, South Texans’ Property Rights Association President; Karnes, Bee and Live Oak

County Landowner

Dr. J. Alfonso “Poncho” Ortega, President of the Society for Range Management

Ellen Randall – South Texans’ Property Rights Association Director, Medina County Landowner

Jenny Sanders – Conservationist, Trinity County Landowner

Robert Sanders – Wildlife Biologist, Trinity County Landowner

Andrew Sansom - Professor of Practice in Geography and Executive Director; The Meadows

Center for Water and the Environment

Neil Shelton – Hartley & Oldham County Landowner

John Shepperd, Texas Foundation for Conservation

Dr. Nova J. Silvy - Fellow and Past President, The Wildlife Society; Aldo Leopold Memorial

Award Recipient

Greg Simons – Wildlife Biologist, Liberty, Jasper, Brazoria and Johnson County Landowner

South Texans’ Property Rights Association

Dr. Don Steinbach – Certified Wildlife Biologist, Executive Director – Texas Chapter of The

Wildlife Society, Washington County Landowner

Tye Stephens - Wildlife Biologist, Ranch Broker

Romey Swanson – Certified Wildlife Biologist; President, Texas Chapter of The Wildlife

Society

Ellen Temple – East Texas Landowner, Leopold Conservation Award Recipient

Tamara Trail – Conservationist, Shackelford County Landowner, Lone Star Land Steward

Award Recipient

Gary Valentine

Dr. Matt Wagner, Certified Wildlife Biologist

Larry Weishuhn – Wildlife Biologist; Co-Founder, Texas Wildlife Association

Irvin Welch – Wildlife Biologist, Landowner

Dr. Neal Wilkins – Certified Wildlife Biologist; South Texans’ Property Rights Association

Director; Professional Member, Boone & Crockett Club

Charlie Williams – Bandera and Medina County Landowner

Simon Winston – Trinity, Angelina, and Nacogdoches County Landowner; Leopold

Conservation Award Recipient

Carl Young - Williamson, Travis, and Brewster County Landowner

CC: The Honorable Greg Abbott, Governor of Texas

The Honorable Dan Patrick, Lieutenant Governor of Texas

The Honorable Dade Phelan, Speaker – Texas House of Representatives

The Honorable Ken King, Chair – House Culture, Recreation & Tourism Committee

The Honorable Tracy King, Chair – House Agriculture & Livestock Committee

The Honorable Charles Perry, Chair – Senate Agriculture, Water & Rural Affairs

Committee

The Honorable James E. Abell, TPW Commissioner

The Honorable Oliver J. Bell, TPW Commissioner

The Honorable Paul L. Foster, TPW Commissioner

The Honorable Anna B. Gallo, TPW Commissioner

The Honorable Jeffery D. Hildebrand, TPW Commissioner

The Honorable Robert L. “Bobby” Patton, Jr., TPW Commissioner

The Honorable Travis B. “Blake” Rowling, TPW Commissioner

The Honorable Richard “Dick” Scott, TPW Commissioner

The Honorable Lee M. Bass, Chairman-Emeritus – TPW Commission

The Honorable T. Dan Friedkin, Chairman-Emeritus – TPW Commission

The Honorable Coleman Locke, Chair - TAHC Commission

The Honorable Jim Eggleston, TAHC Commissioner

The Honorable Jimmie Ruth Evans, TAHC Commissioner

The Honorable Melanie Johnson, TAHC Commissioner

The Honorable Kenneth “Ken” Jordan, TAHC Commissioner

The Honorable Barret J. Klein, TAHC Commissioner

The Honorable Wendee C. Langdon, TAHC Commissioner

The Honorable Joe Leathers, TAHC Commissioner

The Honorable Thomas “Tommy” Oates, TAHC Commissioner

The Honorable Joseph “Joe” Osterkamp, TAHC Commissioner

The Honorable Keith M. Staggs, TAHC Commissioner

The Honorable Leo Vermedahl, TAHC Commissioner

The Honorable Michael L. Vickers, TAHC Commissioner

The Honorable Jimmie Ruth Evans, Chair, TPWD Private Lands Advisory Committee

Mr. Carter Smith, TPWD Executive Director

Dr. Andy Schwartz, DVM, TAHC Executive Director

=====end=====

Terry S. Singeltary Sr.

***> 2nd USA should declare a Declaration of Extraordinary Emergency due to CWD, and all exports of cervid and cervid products must be stopped internationally, and there should be a ban of interstate movement of cervid, until a live cwd test is available.

CONCERNING!

SATURDAY, MAY 29, 2021

Second passage of chronic wasting disease of mule deer to sheep by intracranial inoculation compared to classical scrapie

''Given the results of this study, current diagnostic techniques would be unlikely to distinguish CWD in sheep from scrapie in sheep if cross-species transmission occurred naturally.''

https://scrapie-usa.blogspot.com/2021/05/second-passage-of-chronic-wasting.html

FRIDAY, APRIL 22, 2016

Texas Scrapie Confirmed in a Hartley County Sheep where CWD was detected in a Mule Deer

http://scrapie-usa.blogspot.com/2016/04/texas-scrapie-confirmed-in-hartley.html

For Immediate Release

May 14, 2021

Chronic Wasting Disease Discovered at Deer Breeding Facilities in Matagorda and Mason Counties

AUSTIN, TX – Chronic Wasting Disease (CWD) has been discovered in deer breeding facilities in both Matagorda and Mason counties. This marks the first positive detection of the disease in each county.

An epidemiological investigation found that both deer breeding facilities had received deer from the Uvalde County premises confirmed positive with CWD on March 29, 2021. Postmortem tissue samples were submitted by the permitted deer breeders to assist Texas Parks and Wildlife Department (TPWD) and Texas Animal Health Commission (TAHC) with the epidemiological investigation. The National Veterinary Services Laboratory (NVSL) in Ames, Iowa, has since confirmed CWD in those tissue samples.

TPWD and TAHC officials have taken immediate action to secure all cervids at the Matagorda County and Mason County deer breeding facilities and plan to conduct additional investigations for CWD. In addition, other breeding facilities and release sites that have received deer from these facilities or shipped deer to these facilities during the last five years have been contacted by TPWD and cannot move or release deer at this time.

On March 31, 2021, TPWD and TAHC reported two CWD confirmations at breeding facilities in both Hunt and Uvalde counties. The Hunt facility underwent further DNA testing to confirm animal identification and origin, and on May 12 the DNA test results confirmed the deer’s connection to the premises.

TPWD and TAHC continue to work together to determine the extent of the disease within all the affected facilities and evaluate risks to Texas’ free ranging deer populations. Quick detection of CWD can help mitigate the disease’s spread.

“Regrettably, the gravity of this situation continues to mount with these new CWD positive discoveries, as well as with the full understanding of just how many other facilities and release sites across Texas were connected to the CWD positive sites in Uvalde and Hunt Counties,” said Carter Smith, Executive Director of TPWD. “Along with our partners at the Texas Animal Health Commission, we will continue to exercise great diligence and urgency with this ongoing investigation. Accelerating the testing at other exposed facilities will be critical in ensuring we are doing all we can to arrest the further spread of this disease, which poses great risks to our native deer populations, both captive and free-ranging alike.”

CWD was first recognized in the U.S. in 1967 and has since been documented in captive and/or free-ranging deer in 26 states and 3 Canadian provinces.

In Texas, the disease was first discovered in 2012 in free-ranging mule deer along a remote area of the Hueco Mountains near the Texas-New Mexico border and has since been detected in 228 captive or free-ranging cervids, including white-tailed deer, mule deer, red deer and elk in 13 Texas counties. For more information on previous detections visit the CWD page on the TPWD website. CWD is a fatal neurological disease found in certain cervids, including deer, elk, moose and other members of the deer family. CWD is a slow and progressive disease. Due to a long incubation, cervids infected with CWD may not produce any visible signs for a number of years after becoming infected. As the disease progresses, animals with CWD show changes in behavior and appearance. Clinical signs may include, progressive weight loss, stumbling or tremors with a lack of coordination, excessive thirst, salivation or urination, loss of appetite, teeth grinding, abnormal head posture, and/or drooping ears. To date there is no evidence that CWD poses a risk to humans or non-cervids. However, as a precaution, the U.S. Centers for Disease Control and the World Health Organization recommend not to consume meat from infected animals. For more information about CWD, visit the TPWD web site or the TAHC web site.

###

https://www.tahc.texas.gov/news/2021/2021-05-14_CWD.pdf

SUNDAY, MAY 23, 2021

TEXAS 267 DIFFERENT SITES HAVE RECEIVED DEER FROM AT LEAST ONE OF THE TWO RECENT CWD POSITIVE FACILITIES

https://chronic-wasting-disease.blogspot.com/2021/05/texas-267-different-sites-have-received.html

https://transmissiblespongiformencephalopathy.blogspot.com/2021/05/texas-267-different-sites-have-received.html

Texas Chronic Wasting Disease CWD TSE Prion Positives Mounting 224 To Date

see the latest positives;

2021-04-27 Breeder Deer Mason Facility #10 White-tailed Deer M 2.482191781

2021-04-27 Breeder Deer Uvalde Facility #7 White-tailed Deer M 1.5

2021-04-20 Breeder Deer Matagorda Facility #9 White-tailed Deer F 1.5

2021-03-29 Breeder Deer Uvalde Facility #7 White-tailed Deer F 3.536986301

2021-03-29 Breeder Deer Uvalde Facility #7 White-tailed Deer M 2.178082192

2021-03-29 Breeder Deer Uvalde Facility #7 White-tailed Deer M 3.5

2021-03-29 Breeder Deer Uvalde Facility #7 White-tailed Deer M 1.545205479

2021-03-29 Breeder Deer Uvalde Facility #7 White-tailed Deer M 2.482191781

2021-03-29 Breeder Deer Hunt Facility #8 White-tailed Deer F 2.482191781

https://tpwd.texas.gov/huntwild/wild/diseases/cwd/tracking/

Texas Breeder Deer May Have Spread Brain Disease Into The Wild

ENVIRONMENT 06/01/2021 10:36 am ET Updated 1 day ago

Texas Breeder Deer May Have Spread Brain Disease Into The Wild

Officials are struggling to locate deer sold from facilities infected with chronic wasting disease.

headshot

By Roque Planas

AUSTIN, Texas — State wildlife officials are struggling to trace and halt the spread of deer infected with a contagious brain disease after breeders sold potentially infected animals to hundreds of buyers and released them on game ranches across the state.

Deer at three breeding facilities tested positive for chronic wasting disease in March. Two sites are in the county of Uvalde, west of San Antonio, and owned by the same breeder, while the third is in Hunt County, outside Dallas. Two more facilities that received deer from the Uvalde sites have had positive cases since then, bringing the total of known infected deer to 10 so far.

Officials don’t know how many infected animals the breeders might have sold. Deer breeding is a major business in Texas, where customers will often pay private ranches enclosed by high fencing $10,000 or more to hunt bucks created with the help of artificial insemination, captive rearing and supplemental feed.

The spread of CWD could have severe implications for the state’s wildlife. The disease causes fatal neurodegeneration in cervids like deer, elk and moose.

The state’s tracing effort has identified 267 sites that received deer from what have grown to five facilities with positive results — including 101 sites where deer bred in captivity were released.

High fences block movements in and out of the game ranches that normally buy and release deer. But it’s not uncommon for deer to escape, either by making it over the high fencing or getting past it when damaged. Severe weather, like the February winter storm, can bring down fencing.

That raises the possibility that the disease could have spread from captive deer to wild ones across the state, said Mitch Lockwood, big game program director for the Texas Parks and Wildlife Department.

“That’s what keeps me up at night,” Lockwood told HuffPost. “We hope and pray that didn’t happen. But we can’t find those deer.”

More than half of the animals traced back to the original CWD outbreaks remain untested, Lockwood said. In some cases, state officials are waiting for pending test results before asking the breeder to test suspect deer. In a few cases, breeders have refused to test them, hoping to buy enough time for their fawns to drop first.

Testing for CWD usually requires extracting lymph nodes or brain stem tissue from a carcass. In most cases, buyers have to kill the animals they bought to check for the disease, though live testing is becoming increasingly available.

Those delays could make it easier for CWD to spread. The state requires quarantine for deer exposed to the disease. But if infected animals moved from any of the sites that have yet to submit their tests, they could expose deer that can still legally move around the state.

One reason for the delay is that the state used to let deer breeders batch tissue samples and send them all in ahead of renewing their breeding licenses at the end of the year. The Texas Parks and Wildlife Commission changed that last year, instead requiring samples be sent in within two weeks of a deer’s death.

But the change only took effect in March, shortly before the first positive tests came back. By then, hundreds of potentially exposed deer had already spent months moving across the states and onto game ranches.

Some say officials’ efforts haven’t gone far enough. Rancher Brian Treadwell petitioned the Texas Parks and Wildlife Department last week, demanding a special commission meeting to consider stopping all deer movements.

“You can’t put up a containment zone around these sites anymore,” Treadwell told HuffPost. “I don’t think moving them around is such a good idea anymore.”

An Incurable Disease That Tends To Spread

Like mad cow disease in cattle or Creutzfeldt-Jakob disease in humans, chronic wasting disease causes brain proteins called prions to misfold, leading to a slow, painful death.

It’s unclear whether the disease can jump to humans, like mad cow can. The Centers for Disease Control and Prevention recommends against eating CWD-positive deer meat.

Wildlife biologists consider the disease one of the most severe threats to the country’s deer herds. Once it takes root in a population, wildlife agencies have no method for removing it. Instead, they hope to contain it — a strategy that usually involves reducing herd size and killing off more of the older bucks, among whom the illness usually concentrates.

CWD first appeared in Texas back in 2012 in free-ranging mule deer near the New Mexico border. Since then, the state has identified 66 wild deer infected with the disease in seven counties across the state.

More than 70% of the state’s 224 positive tests over the last nine years occurred either among captive deer or at release sites. CWD was identified in breeder deer at release sites in Medina County, west of San Antonio, a year before free-ranging deer first tested positive there in 2017. Genetic testing later showed that the infected free-ranging deer appeared more closely related to nearby captive animals than wild ones.

The recent spate of CWD cases and possible spread to the wild has fueled long-standing concerns about the controversial deer breeder business in Texas.

Texas is one of a dozen states that allows private citizens to breed deer, but classify them as state-managed wildlife, according to a 2018 report from the Quality Deer Management Association (now the National Deer Association). Most states classify captive deer as livestock. Nearly 1,000 Texans are licensed to breed deer.

Selectively breeding deer and raising them in captivity allows breeders to create bucks with bigger bodies and antlers, driving higher prices at private hunting operations that use them.

The expansion of privatized hunting of artificially bred deer over the last two decades has given many ranches an opportunity to stay intact and economically viable — an ecological win in a state where around 95% of land is privately held and large holdings tend to get subdivided over time. The acreage of many game ranches far exceeds a typical whitetail deer’s range.

But most conservation groups oppose the artificial manipulation of deer herds and view the high fences blocking their movements as an effective privatization of wildlife, which is managed in the United States as a public resource.

Concentrating Animals

While captive deer are no more or less susceptible to CWD than wild ones, critics have long contended that deer breeders spread disease by concentrating animals together, then moving them across distances far greater than they would range if left to wander freely.

What remains unclear is how CWD entered breeder facilities in the first place. None of the breeder facilities had received deer from out of state for six years, according to Lockwood.

It’s unlikely that CWD spread from free-ranging deer into the breeder pens. A wild deer would first have to jump a high fence to get onto the breeder’s property, and then jump a second one to get into the pen.

One possibility is that CWD spread to the facilities through dead deer instead of live ones. Diseased prions can travel on the carcass of a cervid killed elsewhere, like when a hunter travels to an area where the disease is present and brings meat home.

Raising awareness among any individuals who move deer and their carcasses ― whether breeders, live trappers or hunters ― is the best way to check CWD’s spread, according to Lockwood.

“It is unquestionably the biggest threat facing North American deer,” Lockwood said. “And it will only get worse if it spreads.”

https://www.huffpost.com/entry/texas-deer-breeders-cwd_n_60b55044e4b0f2a82eed2d52

Politicians and ANIMAL FREAK SHOWS IN TEXAS AND CWD

ALSO, IT'S ALWAYS A FREAK SHOW IN TEXAS, IF POLITICIANS ARE NOW JERKING DEER OFF FOR CAMPAIGN MONEY, THEY ARE NOW CLONING DEER, WHAT NEXT;

WEDNESDAY, APRIL 21, 2021

***> A Texas Rancher Cloned Deer For Years. Some Lawmakers Want To Legalize It (what about cwd tse prion)? <***

https://chronic-wasting-disease.blogspot.com/2021/04/a-texas-rancher-cloned-deer-for-years.html

WHAT COULD GO WRONG, ASK BAMBI;

https://www.youtube.com/watch?v=JPgxCSeXtpA&list=PLiZxWe0ejyv_2eA9Z26DnHqiImlkcTSIl

TUESDAY, DECEMBER 31, 2019

In Vitro detection of Chronic Wasting Disease (CWD) prions in semen and reproductive tissues of white tailed deer bucks (Odocoileus virginianus

SUNDAY, AUGUST 02, 2015

TEXAS CWD, Have you been ThunderStruck, deer semen, straw bred bucks, super ovulation, and the potential TSE Prion connection, what if?

https://chronic-wasting-disease.blogspot.com/2019/12/in-vitro-detection-of-chronic-wasting.html

SUNDAY, FEBRUARY 16, 2020

***> Jerking for Dollars, Are Texas Politicians and Legislators Masturbating Deer For Money, and likely spreading CWD TSE Prion?

https://chronic-wasting-disease.blogspot.com/2020/02/jerking-for-dollars-are-texas.html

http://chronic-wasting-disease.blogspot.com/2014/07/supercalifragilisticexpialidocious-or.html

***> 181 CWD-trace facilities associated with the CWD-positive deer breeding facilities in Hunt and Uvalde Counties, and some are out of state/country in Mexico

i finally got a recent copy of the CWD-trace facilities associated with the CWD-positive deer breeding facilities in Hunt and Uvalde Counties.

seems to date, there are 181 CWD-trace facilities associated with the CWD-positive deer breeding facilities in Hunt and Uvalde Counties, and some are out of state/country in Mexico. i was told that in the coming weeks, some of the facilities will start testing for cwd, and those results will be forthcoming later on. i hope they don't flounder on depopulation efforts if any positives are found. sad for Mexico (8 facilities).

Chronic Wasting Disease Discovered at Deer Breeding Facilities in Hunt and Uvalde Counties

MARCH 31, 2021

https://tpwd.texas.gov/newsmedia/releases/?req=20210331b

Texas Confirms CWD TSE Prion in 213 white-tailed deer, mule deer, red deer and elk to date, 148 connected to deer breeding facilities and release sites.

https://tpwd.texas.gov/newsmedia/releases/?req=20210301b

TUESDAY, APRIL 13, 2021

Implications of farmed-cervid movements on the transmission of chronic wasting disease

Conclusion

In conclusion, given that CWD transmission can occur through contact with infected body parts or through indirect contacts via contamination of feed and other fomites, understanding animal movements is critical for mitigating disease spread. Long distance commercial movements of cervids pose one risk for spread of CWD. This study approach can be used to understand disease transmission risks across the region and in North America in general.

https://www.sciencedirect.com/science/article/abs/pii/S0167587720301537?via%3Dihub

TUESDAY, APRIL 13, 2021

Implications of farmed-cervid movements on the transmission of chronic wasting disease

https://chronic-wasting-disease.blogspot.com/2021/04/implications-of-farmed-cervid-movements.html

WEDNESDAY, MARCH 31, 2021

Texas TPWD TAHC Chronic Wasting Disease Discovered at Deer Breeding Facilities in Hunt and Uvalde Counties

https://chronic-wasting-disease.blogspot.com/2021/03/texas-tpwd-tahc-chronic-wasting-disease.html

THURSDAY, MARCH 25, 2021

Texas CWD suspect positive results for a couple of deer breeding facilities

https://chronic-wasting-disease.blogspot.com/2021/03/texas-cwd-suspect-positive-results-for.html

TUESDAY, MARCH 02, 2021

Texas Confirms CWD TSE Prion in 213 white-tailed deer, mule deer, red deer and elk to date, 148 connected to deer breeding facilities and release sites

https://chronic-wasting-disease.blogspot.com/2021/03/texas-confirms-cwd-tse-prion-in-213.html

***> TEXAS BREEDER DEER ESCAPEE WITH CWD IN THE WILD, or so the genetics would show?

OH NO, please tell me i heard this wrong, a potential Texas captive escapee with cwd in the wild, in an area with positive captive cwd herd?

apparently, no ID though. tell me it ain't so please...

23:00 minute mark

''Free Ranging Deer, Dr. Deyoung looked at Genetics of this free ranging deer and what he found was, that the genetics on this deer were more similar to captive deer, than the free ranging population, but he did not see a significant connection to any one captive facility that he analyzed, so we believe, Ahhhhhh, this animal had some captive ahhh, whatnot.''

https://youtu.be/aoPDeGL6mpQ?t=1384

TEXAS CWD STRAIN

77. Assessing chronic wasting disease strain differences in free-ranging cervids across the United States

Kaitlyn M. Wagnera, Caitlin Ott-Connb, Kelly Strakab, Bob Dittmarc, Jasmine Battend, Robyn Piercea, Mercedes Hennessya, Elizabeth Gordona, Brett Israela, Jenn Ballarde and Mark D Zabela

aPrion Research Center at Colorado State University; bMichigan Department of Natural Resources; cTexas Parks and Wildlife Department; dMissouri Department of Conservation, 5. Arkansas Game and Fish Commission CONTACT Kaitlyn M. Wagner miedkait@rams.colostate.edu

ABSTRACT

Background/Introduction: Chronic wasting disease (CWD) is an invariably fatal prion disease affecting captive and free-ranging cervids, including white-tailed deer, mule deer, moose, elk, and reindeer. Since the initial description of the disease in the 1960’s, CWD has spread to 23 states, 3 Canadian Provinces, South Korea, Norway and, most recently, Finland. While some outbreaks of CWD were caused by transport of infected animals from endemic regions, the origin of CWD in other epizootics is unclear and has not been characterized. Previous studies have shown that there are two distinct strains of CWD. However, the continuous spread and the unclear origin of several outbreaks warrant continued surveillance and further characterization of strain diversity.

Materials and Methods: To address these knowledge gaps, we used biochemical tests to assess strain differences between CWD outbreaks in Michigan, Texas, Missouri, and Colorado, USA. Brain or lymph node samples were homogenized and digested in 50 µg/mL proteinase K (PK). These samples were then run on a Western blot to assess glycoform ratio and electrophoretic mobility. Texas samples were digested in 100 µg/mL PK. To assess conformational stability, brain or lymph node homogenates were incubated in increasing concentrations of guanidine hydrochloride from 0 M to 4 M in 0.5 M increments. Samples were then precipitated in methanol overnight, washed and PK digested in 50 µg/mL PK before slot blotting.

Results: Our results have found significant differences in glycoform ratio between CWD from Michigan and Colorado, but no differences were observed in conformational stability assays. Interestingly, when testing our CWD isolates from Texas to analyse electrophoretic mobility and glycoform ratio, we found that these samples did not exhibit the characteristic band shift when treated with PK, but PK resistant material remained. Additionally, results from our conformational stability assay demonstrate a unique profile of these Texas isolates. Testing of samples from Missouri is currently underway.

Conclusions: Thus far, our data indicate that there are strain differences between CWD circulating in Michigan and CWD in Colorado and provide important insight into CWD strain differences between two non-contiguous outbreaks. We have also identified a unique strain of CWD in Texas with biochemical strain properties not seen in any of our other CWD isolates. These results highlight the importance of continued surveillance to better understand this devastating disease. These results have important implications for CWD emergence, evolution and our understanding of prion strain heterogeneity on the landscape.

https://www.tandfonline.com/doi/full/10.1080/19336896.2019.1615197

The disease devastating deer herds may also threaten human health

Scientists are exploring the origins of chronic wasting disease before it becomes truly catastrophic.

Rae Ellen Bichell

Image credit: David Parsons/Istock

April 8, 2019

This story was published in collaboration with the Mountain West News Bureau, a collaboration between Wyoming Public Media, Boise State Public Radio in Idaho, KUER in Salt Lake City and KRCC and KUNC in Colorado.

SNIP...

One day in late February, in their laboratory in Fort Collins, Colorado, Wagner and Zabel compared the prions from the brains of CWD-infected deer in Texas with those of elk in Colorado. They want to know if the proteins were all mangled in the same way, or not. “If they are different, this would suggest that we have different strain properties, which is evidence as we're building our case that we might have multiple strains of CWD circulating in the U.S.,” says Wagner.

Step one is to see if they’re equally easy to destroy using a chemical called guanidine. The shape of a prion dictates everything, including the way it interacts with an animal’s cells and the ease with which chemicals can unfold it.

“Moment of truth,” said Wagner, as she and Zabel huddled around a computer, waiting for results to come through. When they did, Zabel was surprised.

“Wow,” he said. “Unlike anything we've seen before.”

The prions from the Texas deer were a lot harder to destroy than the ones from the Colorado elk. In fact, the guanidine barely damaged them at all. “We’ve never seen that before in any prion strain, which means that it has a completely different structure than we've ever seen before,” says Zabel. And that suggests that it might be a very different kind of chronic wasting disease. The researchers ran the same test on another Texas deer, with the same results.

Now, these are only the preliminary results from a few animals. Wagner and Zabel have a lot more experiments to do. But if future tests come to the same conclusion, it would support their hypothesis that there are multiple strains of chronic wasting disease out there, all with different origins. That, in turn, could mean that this disease will become even trickier to manage than it already is.

And, Zabel adds, there’s something else. “If it's still evolving, it may still evolve into a form that could potentially, eventually affect humans,” he says.

Zabel is not the only one worried about that possibility.

OSTERHOLM, THE EPIDEMIOLOGIST from Minnesota, is also concerned. He directs the Center for Infectious Disease Research and Policy at the University of Minnesota, and is serving a one-year stint as a “Science Envoy for Health Security” with the U.S. State Department. In February, he told Minnesota lawmakers that when it comes to chronic wasting disease, we are playing with fire. “You are going to hear from people that this is not going to be a problem other than a game farm issue. You're going to hear from people that it's not going to transmit to people, and I hope they're right, but I wouldn't bet on it,” he said. “And if we lose this one and haven’t done all we can do, we will pay a price.”

If that wasn’t warning enough, he added: “Just remember what happened in England.”

He was talking about mad cow disease. Decades ago, Osterholm got involved in studying the potential for the newly emerging condition — bovine spongiform encephalopathy, or BSE for short — to be transmitted to humans.

At that point, researchers had yet to document a prion disease in animals that could infect people. They did, however, have a few pieces of the puzzle. For one, work in Papua New Guinea had shown that people could transmit prion diseases to each other if they practiced cannibalism, especially of the brain-eating variety. They also knew that BSE was spreading quickly between cattle. Osterholm says he and others worried that the more widespread it became, the more chances it might have to change into something that could sicken people.

“A lot of people thought that it was an overreaction,” says Osterholm. “Then, of course, in 1996, 10 years later, we recognized that in fact transmission had occurred.” Variant Creutzfeldt-Jakob disease, as the illness is called when it appears in human beings, has infected about 230 people worldwide. Osterholm says he feels like he’s having déjà vu, except that instead of mad cow, now it’s chronic wasting disease that’s spreading in animals, with the potential to cross the species barrier to infect humans.

But some view Osterholm’s statements as pure fear-mongering.“To say that without any concrete proof — I think that's irrational,” says Daniel Schmidt, the editor-in-chief of Deer & Deer Hunting. He says Osterholm needs to lower the fear flag. “If CWD is a threat, it is more to the lifestyle of the hunting public in America,” says Schmidt. “If you scare people enough in America, they're going to stop doing something.”

Schmidt and his family, who live in Wisconsin, eat wild venison almost every day, and he says they don’t give chronic wasting disease a second thought. If you need something to worry about, he says, how about climate change, or pesticides in your strawberries? “This is not a zombie apocalypse, and the hamster wheel of fear-mongering is nothing short of sensationalism, in my opinion,” Schmidt says.

So, who’s right? Could chronic wasting disease present a public health crisis? Or are we, as Schmidt put it, merely hamsters spinning the wheel of fear?

The answer to that question may largely depend on Stefanie Czub, a professor of veterinary medicine with the University of Calgary. Czub runs the Canadian Food Inspection Agency lab that tests for mad cow disease, and everyone is waiting for results from her decade-long study of chronic wasting disease in macaque monkeys, which is scheduled to end in March 2020.

“At this point, what we would like to stress — my collaborators and I — is that we have some evidence that it might infect non-human primates.”

While Czub cautions that the project isn’t yet complete, she does have some preliminary results: “At this point, what we would like to stress — my collaborators and I — is that we have some evidence that it might infect non-human primates.”

Czub and her collaborators exposed 18 macaque monkeys to chronic wasting disease prions. Some had the prions inserted straight into their brains. Some ate infected venison, while others were exposed via blood transfusion. And some were given little cuts that were wrapped in infected deer brain, which was meant to model how a hunter might be exposed to infectious viscera after getting nicked during field dressing. There were also three control animals, which were exposed to healthy deer and elk tissue.

So far, four out of the 18 monkeys developed what Czub calls “subtle and transient” symptoms that “could be indicative” of chronic wasting disease. Two of those animals had received CWD straight into their brains. Two had eaten infected meat.

Those four lost weight and became anxious. “Anxiety is a very common clinical expression in animal prion diseases,” says Czub. “It is one of the main symptoms in bovine spongiform encephalopathy, and that is the reason why some people decided to call it ‘mad cow disease.’ The animals are not mad, they are scared to death.” In monkeys, that involves crouching in the farthest corner of the cage. Czub says they shivered and had difficulties picking up pieces of food. One monkey lost a third of its body weight in just six months.

After the four symptomatic animals were euthanized, Czub and her colleagues ran a bunch of tests, which Czub says “suggested the presence of CWD.” But there are a number of factors that make this complicated. First off, three of the four sick monkeys also happened to have diabetes. “And it's really important to mention that, because diabetes — uncontrolled diabetes — really does induce wasting, so we need we need to be super careful in the interpretation of wasting,” says Czub.

Czub has presented her preliminary results at conferences, but they have not yet gone through the true scientific ringer: peer-reviewed publication. That’s a crucial step, because where one researcher might see an unusual level of anxiety, another might just see an animal in captivity and under stress. Even the results from more technical evaluations, like analyzing slices of the brain for neuron death, could be interpreted in different ways. “We'd like to see them published so we can get a better idea of how strong the data really is to support transmission,” says Brent Race, a staff scientist at Rocky Mountain Laboratories in Montana, which is part of the National Institutes of Health’s National Institute of Allergy and Infectious Diseases.

Race and his colleagues ran an experiment similar to Czub’s, in which they gave macaques deer and elk brain tainted with chronic wasting disease, in some cases injected into the monkeys’ brains and in others delivered to the stomach through a tube. In behavior and biochemical tests, the animals appeared no different from those in the control group.“We watched our macaque monkeys for over 13 years in some cases, and we were unable to find any evidence of transmission of chronic wasting disease,” says Race. A later study on “humanized” mice spliced with a human gene also showed no strong proof of transmission, despite the fact that Race and his colleagues tried to make it as easy as possible for the infection to take hold.

In a different study, however, Race’s research concluded that a different type of primate — squirrel monkeys — were highly susceptible to chronic wasting disease. Thirteen squirrel monkeys were exposed to the disease directly in their brains. Every one of them developed symptoms, including severe weight loss, tremors, drooling and weakness, after an average of about 4 years. The researchers fed another group of 12 squirrel monkeys infected meat and found that 11 of them developed chronic wasting disease an average of about half a dozen years after exposure. A systematic review of 23 studies cited the squirrel monkey findings as a reason that human infection “cannot be entirely ruled out.”

Still, human transmission remains uncertain. As the researchers noted in the journal Emerging Infectious Diseases, macaque monkeys are biologically much closer to humans than squirrel monkeys. “Aside from a few benchtop assays and the unpublished macaque study from Canada, news has been very encouraging,” says Race.

Christina Sigurdson, a professor of pathology at UC San Diego and UC Davis, did a study that hunting enthusiasts have pointed to as a reason not to worry about chronic wasting disease. It showed that a certain part of human prions makes it hard for chronic wasting disease prions to guide them into misfolding, kind of like how a zipper just won’t zip if there’s a pebble stuck somewhere in its teeth. “It suggested that this region was a barrier — at least, a partial barrier — for blocking infection,” says Sigurdson.

But only a partial barrier — and even then, it’s only against the particular versions of chronic wasting disease that Sigurdson tested from Colorado deer and elk. “We need more research to find out how many strains there are, how different are these different strains and would there potentially be some strains in the U.S. that could be infectious for people,” she says.

The Centers for Disease Control and Prevention has not yet found any evidence of chronic wasting disease in people, despite researchers actively looking for it. Epidemiologists in states like Colorado and Wyoming have also been watching for an elevated rate of prion disease in hunters — hunters like researcher Brent Race.

“I’m an avid hunter myself, and my entire family eats it,” Race says. “Actually, we raise cattle and we sell all of our cattle and eat deer and elk instead.” But Race wouldn’t go so far as to eat meat that hasn’t been tested for chronic wasting disease. That feeling is shared by pretty much every person in this story: If you’re hunting in an area with chronic wasting disease, get the animal tested before it ever hits your plate, and don’t eat meat that tests positive.

“Otherwise,” says Osterholm, “I wish you well and hope you enjoy your venison.”

Rae Ellen Bichell is a regional radio reporter with the Mountain West News Bureau. She’s based at KUNC in northern Colorado. She frequently covers science and health. Email High Country News at editor@hcn.org or submit a letter to the editor.

https://www.hcn.org/articles/wildlife-the-disease-devastating-deer-herds-may-also-threaten-human-health-science

FRIDAY, JUNE 04, 2021

Texas Breeder Deer May Have Spread Brain Disease CWD TSE Prion Into The Wild

https://chronic-wasting-disease.blogspot.com/2021/06/texas-breeder-deer-may-have-spread.html

SUNDAY, JANUARY 22, 2017

Texas 85th Legislative Session 2017 Chronic Wasting Disease CWD TSE Prion Cervid Captive Breeder Industry

http://chronic-wasting-disease.blogspot.com/2017/01/texas-85th-legislative-session-2017.html

FRIDAY, JANUARY 27, 2017

TEXAS, Politicians, TAHC, TPWD, and the spread of CWD TSE Prion in Texas

http://chronic-wasting-disease.blogspot.com/2017/01/texas-politicians-tahc-tpwd-and-spread.html

SUNDAY, MAY 14, 2017

85th Legislative Session 2017 AND THE TEXAS TWO STEP Chronic Wasting Disease CWD TSE Prion, and paying to play $$$

http://chronic-wasting-disease.blogspot.com/2017/05/85th-legislative-session-2017-and-texas.html

Powerful Abbott appointee's lobbying sparks blowback in Legislature

In an ironic twist for Gov. Greg Abbott, who has made ethics reform an urgent political priority, the Texas House is taking aim at what critics call a "pay to play" culture among his appointees.

BY JAY ROOT MAY 12, 2017 12 AM

Houston billionaire Dan Friedkin is chairman of the Texas Parks and Wildlife Commission.

Texas Parks and Wildlife Commission

When Gov. Greg Abbott tapped one of his top campaign donors to become chairman of the Texas Parks and Wildlife Commission, he didn’t get a part-time appointee who would merely draft rules and implement conservation laws passed by the Legislature.

In Dan Friedkin, the governor got a Houston billionaire — with a team of privately funded lobbyists — willing to use his influence to ensure his wildlife interests are taken into account by the Legislature before they pass those laws, interviews and records show.

On the receiving end of that influence, and not in a happy way, is state Rep. Chris Paddie, R-Marshall. Paddie said a lobbyist working for Friedkin’s business empire, which includes a massive South Texas hunting ranch, has been working against his deer breeder management bill, which many large ranchers oppose. The state Parks and Wildlife Department oversees deer breeding regulations in Texas.

“Many times these appointees are well-heeled, very influential people,” Paddie said. “Overall, I feel that it’s inappropriate for an appointee of a board or commission to have personal lobbyists lobbying on issues related to that board or commission.”

Under Texas law, state agencies are barred from lobbying the Legislature. But the powerful people who oversee them aren’t.

If Paddie and dozens of his colleagues get their way, that practice soon will be a Class A misdemeanor.

Last weekend, Paddie attached a ban on appointee lobbying — which would apply to any issues intersecting with their state responsibilities — to an ethics bill that already had powerful friends of the governor in its crosshairs. The provision was adopted unanimously and the bill sailed out of the Texas House on a 91-48 vote Saturday.

The ethics bill, authored by Rep. Lyle Larson, R-San Antonio, would bar big campaign donors from getting appointed by governors in the first place. Anyone who contributed over $2,500 would be barred from serving on state boards and commissions.

Larson pointed to news articles documenting the amount of campaign money appointees have collectively given governors. Last year the San Antonio Express-News calculated that Abbott had received nearly $9 million from people he’s picked for appointed office; before that, a widely cited report from Texans for Public Justice found former Gov. Rick Perry had received $17 million from his own appointees.

Larson said 20 years from now, Texans will be reading the same stories about a future governor unless the Legislature does something about it now.

“We’ve read that article for the last three decades,” Larson said during a brief floor speech. “This is your opportunity to say, 'We need to stop this.' The most egregious ethics violation we’ve got in the state is the pay to play in the governor’s office.”

A prodigious fundraiser, Abbott has put plenty of big donors on prestigious boards and commissions. On the Parks and Wildlife Commission alone, he has installed three mega-donors — pipeline mogul Kelcy Warren, who’s given Abbott more than $800,000 over his statewide political career; Houston businessman S. Reed Morian, who has given $600,000; and Friedkin, who personally donated more than $700,000 — while his Gulf States Toyota PAC gave Abbott another $100,000, according to Ethics Commission records.

Passage of Larson’s HB 3305 represents an ironic twist for Abbott, who for the second session in a row has made ethics reform an urgent political priority — resulting in a bill that's now taking aim at his gubernatorial appointments. Abbott, who has made a habit of ignoring tough questions, hasn't made any public statements about the bill, and his office did not respond to multiple requests for comment.

Friedkin — whose wealth is estimated at $3.4 billion by Forbes — is the owner and CEO of Gulf States Toyota, founded in 1969, which has had the exclusive rights to distribute new Toyotas in Texas and four nearby states. He’d also been a mega-donor to former Gov. Rick Perry, who first appointed Friedkin to the Parks and Wildlife Commission in 2005. Abbott made Friedkin chairman of the commission in 2015.

Requests for comment from Friedkin's office went unanswered.

In addition to his public role as parks and wildlife chairman, a perch that gives him significant influence over deer management issues, Friedkin has private wildlife interests. He owns the sprawling Comanche Ranch in South Texas, according to published news accounts.

The January 2014 edition of Texas Wildlife, published by the Texas Wildlife Association, described Friedkin’s Comanche Ranch as “privately owned and privately hunted” and said it’s “in the business to produce as many trophy bucks as possible, without damaging the native habitat.”

The association, which advocates for private landowners and hunting rights, has locked horns with deer breeding interests at Parks and Wildlife and the Capitol. They compete against each other in the lucrative trophy deer hunting market — and the battle between them perennially spills into the rule-making process at the Parks and Wildlife Commission.

One of their battles centers on how captive deer are tagged so that game wardens and others can distinguish them from native deer. Current law requires a combination of tags and tattoos, and the ranchers and large landowners want to keep it that way. The breeders, meanwhile, favor tagging deer with microchips, which they contend are more accurate and foolproof.

The Wildlife Association said in a Facebook post that removing visible tag or tattoo requirements and allowing microchip tracking “creates real biosecurity risks and blurs ethical lines in the hunting community, as captive deer breeders are allowed to transport and release these animals to be co-mingled with pasture-born deer.” Proponents of the current system say tough rules on breeders are needed to keep out imported deer that may carry Chronic Wasting Disease, which has been found in Texas.

On the other side of the issue is the Texas Deer Association, which represents breeder interests. Executive Director Patrick Tarlton said opposition to his $1.6 billion industry stems less from environmental and health concerns and more from wealthy ranch owners who want to boost profits from trophy-seeking hunters. He notes that Chronic Wasting Disease has been found in both free range and captive deer.

Paddie sided with the breeders by filing House Bill 2855, which would allow breeders to track their deer with microchips instead of relying on physical tags that they say can be torn off.

No one identifying themselves as a Friedkin corporate lobbyist opposed the deer breeding bills during public hearings, according to House and Senate committee records published online.

Behind the scenes, it was a different story.

Paddie said his chief of staff reached out to Laird Doran, one of several lobbyists for Friedkin’s Gulf States Toyota, after hearing that he was trying to convince other legislators to help defeat Paddie's deer microchip bill.

“My chief called him and said, 'Hey, if you’ve got a problem with our bill why aren’t you talking to us?’ ” Paddie said. “He said he represented the Friedkin Group when that happened.”

According to an email from an aide to Sen. Craig Estes, R-Wichita Falls, who is carrying the deer breeding bill in the Senate, Doran also identified himself as a representative of the “Friedkin Group.” That’s the name of the consortium that contains Friedkin's Gulf States Toyota, according to the company’s Linked-In page. He told Estes’ aide that the Friedkin group was opposed to any bill that would “remove requirements for (deer) ear tags,” the senator’s office confirmed.

It’s not clear exactly which Friedkin interests Doran was advancing. Doran is registered at the Texas Ethics Commission with a single entity — Gulf States Toyota — and the agency has no record of a lobbyist working for an entity or individual with the name Friedkin in it, the commission confirmed Wednesday afternoon.

However, Doran checked a variety of non-automotive subject areas in which he is lobbying during this legislative session on behalf of Friedkin’s lucrative distributorship, including “animals,” “parks & wildlife,” “state agencies, boards & commissions,” “environment” and more, his detailed lobby disclosures show.

Doran, director of government relations and senior counsel at the Friedkin Group, did not return phone and email messages left by The Texas Tribune.

Estes said he didn’t have a problem with a governor's appointee engaging in lobbying on issues that affected their private interests, as long as they keep that separate from their state roles.

“I don’t think they should be barred from expressing their views as long as they’re careful to say these are my views, not the views of the agency I’m representing,” Estes said.

But Tarlton, the deer association director, said Friedkin’s use of lobbyists to oppose deer breeders in the Legislature gives the breeders' opponents a huge advantage.

“I think that if the commissioner of Texas Parks and Wildlife is actively lobbying against an industry which his department directly oversees, it absolutely sets up an unfair and closed system of government,” Tarlton said. “The commission is supposed to be the unbiased and equitable oversight for everything wildlife.”

Paddie hopes his amendment to Larsen's ethics bill will even the playing field. He referred to the wealthy Parks and Wildlife chairman (see the 2:29:00 mark in this recorded exchange) when he tacked the appointee-lobbying provision onto Larson’s bill.

Paddie said he’s not singling out anyone. He said it would apply to other powerful gubernatorial appointees in a position to do the same.

“I could have named any number of examples as far as the agencies in particular,” Paddie said. “I want to stop it if anyone serving on any agency is doing this.”

Ryan Murphy contributed to this report.

Disclosure: The Texas Wildlife Association, Texas Parks and Wildlife Department and Gulf States Toyota have been financial supporters of The Texas Tribune. A complete list of Tribune donors and sponsors is available here.

https://www.texastribune.org/2017/05/12/powerful-abbott-appointees-lobbying-sparks-blowback-legislature/

TUESDAY, AUGUST 02, 2016

TEXAS TPWD Sets Public Hearings on Deer Movement Rule Proposals in Areas with CWD Rule Terry S. Singeltary Sr. comment submission

http://chronic-wasting-disease.blogspot.com/2016/08/texas-tpwd-sets-public-hearings-on-deer.html

SUNDAY, MAY 22, 2016

TEXAS CWD DEER BREEDERS PLEA TO GOVERNOR ABBOTT TO CIRCUMVENT TPWD SOUND SCIENCE TO LET DISEASE SPREAD

http://chronic-wasting-disease.blogspot.com/2016/05/texas-cwd-deer-breeders-plea-to.html

Wednesday, May 04, 2016

TPWD proposes the repeal of §§65.90 -65.94 and new §§65.90 -65.99 Concerning Chronic Wasting Disease - Movement of Deer Singeltary Comment Submission

http://chronic-wasting-disease.blogspot.com/2016/05/tpwd-proposes-repeal-of-6590-6594-and_4.html

Terry S. Singeltary Sr. Your opinions and comments have been submitted successfully. Thank you for participating in the TPWD regulatory process.

Wednesday, October 28, 2015

Interim Chronic Wasting Disease Response Rules Comment online through 07:00 a.m. November 5, 2015

http://chronic-wasting-disease.blogspot.com/2015/10/interim-chronic-wasting-disease.html

Texas 84th Legislative Session Sunday, December 14, 2014

*** TEXAS 84th Legislature commencing this January, deer breeders are expected to advocate for bills that will seek to further deregulate their industry

http://chronic-wasting-disease.blogspot.com/2014/12/texas-84th-legislature-commencing-this.html

TUESDAY, DECEMBER 16, 2014

Texas 84th Legislature 2015 H.R. No. 2597 Kuempel Deer Breeding Industry TAHC TPWD CWD TSE PRION

http://chronic-wasting-disease.blogspot.com/2014/12/texas-84th-legislature-2015-hr-no-2597.html

expand this to see all breeder cwd, and then think of what they have released at release sites...

https://tpwd.texas.gov/huntwild/wild/diseases/cwd/tracking/

TAHC Chapter 40, Chronic Wasting Disease Terry Singeltary Comment Submission

https://chronic-wasting-disease.blogspot.com/2021/08/tahc-chapter-40-chronic-wasting-disease.html

***> TEXAS HISTORY OF CWD <***

Singeltary telling TAHC, that CWD was waltzing into Texas from WSMR around Trans Pecos region, starting around 2001, 2002, and every year, there after, until New Mexico finally shamed TAHC et al to test where i had been telling them to test for a decade. 2012 cwd was detected first right there where i had been trying to tell TAHC for 10 years.

***> Singeltary on Texas Chronic Wasting Disease CWD TSE Prion History <***

http://chronic-wasting-disease.blogspot.com/2017/08/texas-chronic-wasting-disease-cwd-tse.html

***> 1st and foremost your biggest problem is 'VOLUNTARY'! AS with the BSE 589.2001 FEED REGULATIONS, especially since it is still voluntary with cervid, knowing full well that cwd and scrapie will transmit to pigs by oral route. VOLUNTARY DOES NOT WORK! all animal products should be banned and be made mandatory, and the herd certification program should be mandatory, or you don't move cervid. IF THE CWD HERD CERTIFICATION IS NOT MANDATORY, it will be another colossal tse prion failure from the start.

***> 2nd USA should declare a Declaration of Extraordinary Emergency due to CWD, and all exports of cervid and cervid products must be stopped internationally, and there should be a ban of interstate movement of cervid, until a live cwd test is available.

***> 3rd Captive Farmed cervid ESCAPEES should be made mandatory to report immediately, and strict regulations for those suspect cwd deer that just happen to disappear. IF a cervid escapes and is not found, that farm should be indefinitely shut down, all movement, until aid MIA cervid is found, and if not ever found, that farm shut down permanently.

***> 4th Captive Farmed Cervid, INDEMNITY, NO MORE Federal indemnity program, or what i call, ENTITLEMENT PROGRAM for game farm industry. NO MORE BAIL OUTS FROM TAX PAYERS. if the captive industry can't buy insurance to protect not only themselves, but also their customers, and especially the STATE, from Chronic Wasting Disease CWD TSE Prion or what some call mad deer disease and harm therefrom, IF they can't afford to buy that insurance that will cover all of it, then they DO NOT GET A PERMIT to have a game farm for anything. This CWD TSE Prion can/could/has caused property values to fall from some reports in some places. roll the dice, how much is a state willing to lose?

***> 5th QUARANTINE OF ALL FARMED CAPTIVE, BREEDERS, URINE, ANTLER, VELVET, SPERM, OR ANY FACILITY, AND THEIR PRODUCTS, that has been confirmed to have Chronic Wasting Disease CWD TSE Prion, the QUARANTINE should be for 21 years due to science showing what scrapie can do. 5 years is NOT near long enough. see; Infectious agent of sheep scrapie may persist in the environment for at least 16 to 21 years.

***> 6th America BSE 589.2001 FEED REGULATIONS CWD TSE Prion

***> 7TH TRUCKING TRANSPORTING CERVID CHRONIC WASTING DISEASE TSE PRION VIOLATING THE LACEY ACT

***> 8TH ALL CAPTIVE FARMING CERVID OPERATIONS MUST BE INSURED TO PAY FOR ANY CLEAN UP OF CWD AND QUARANTINE THERE FROM FOR THE STATE, NO MORE ENTITLEMENT PROGRAM FOR CERVID GAME FARMING PAY TO PLAY FOR CWD TSE PRION OFF THE TAX PAYERS BACK.

***> 9TH ANY STATE WITH DOCUMENTED CWD, INTERSTATE, NATIONAL, AND INTERNATIONAL MOVEMENT OF ALL CERVID, AND ALL CERVID PRODUCTS MUST BE HALTED!

***> 10TH BAN THE SALE OF STRAW BRED BUCKS AND ALL CERVID SEMEN AND URINE PRODUCTS

***> 11th ALL CAPTIVE FARMED CERVID AND THEIR PRODUCTS MUST BE CWD TSE PRION TESTED ANNUALLY AND BEFORE SALE FOR CWD TSE PRION

SEE FULL SCIENCE REFERENCES AND REASONINGS ;

Control of Chronic Wasting Disease OMB Control Number: 0579-0189 APHIS-2021-0004 Singeltary Submission

https://www.regulations.gov/comment/APHIS-2021-0004-0002

https://downloads.regulations.gov/APHIS-2021-0004-0002/attachment_1.pdf

Docket No. APHIS-2018-0011 Chronic Wasting Disease Herd Certification

https://www.regulations.gov/document/APHIS-2018-0011-0003

https://downloads.regulations.gov/APHIS-2018-0011-0003/attachment_1.pdf

5 or 6 years quarantine is NOT LONG ENOUGH FOR CWD TSE PRION !!!

QUARANTINE NEEDS TO BE 21 YEARS FOR CWD TSE PRION !

FRIDAY, APRIL 30, 2021

Should Property Evaluations Contain Scrapie, CWD, TSE PRION Environmental Contamination of the land?

***> Confidential!!!!

***> As early as 1992-3 there had been long studies conducted on small pastures containing scrapie infected sheep at the sheep research station associated with the Neuropathogenesis Unit in Edinburgh, Scotland. Whether these are documented...I don't know. But personal recounts both heard and recorded in a daily journal indicate that leaving the pastures free and replacing the topsoil completely at least 2 feet of thickness each year for SEVEN years....and then when very clean (proven scrapie free) sheep were placed on these small pastures.... the new sheep also broke out with scrapie and passed it to offspring. I am not sure that TSE contaminated ground could ever be free of the agent!! A very frightening revelation!!!

---end personal email---end...tss

https://transmissiblespongiformencephalopathy.blogspot.com/2021/04/should-property-evaluations-contain.html

WEDNESDAY, DECEMBER 04, 2013

Chronic Wasting Disease CWD and Land Value concerns?

http://chronic-wasting-disease.blogspot.com/2013/12/chronic-wasting-disease-cwd-and-land.html

***> This is very likely to have parallels with control efforts for CWD in cervids.

Paper

Rapid recontamination of a farm building occurs after attempted prion removal

Kevin Christopher Gough BSc (Hons), PhD Claire Alison Baker BSc (Hons) Steve Hawkins MIBiol Hugh Simmons BVSc, MRCVS, MBA, MA Timm Konold DrMedVet, PhD, MRCVS … See all authors

First published: 19 January 2019 https://doi.org/10.1136/vr.105054

Abstract

The transmissible spongiform encephalopathy scrapie of sheep/goats and chronic wasting disease of cervids are associated with environmental reservoirs of infectivity. Preventing environmental prions acting as a source of infectivity to healthy animals is of major concern to farms that have had outbreaks of scrapie and also to the health management of wild and farmed cervids. Here, an efficient scrapie decontamination protocol was applied to a farm with high levels of environmental contamination with the scrapie agent. Post‐decontamination, no prion material was detected within samples taken from the farm buildings as determined using a sensitive in vitro replication assay (sPMCA). A bioassay consisting of 25 newborn lambs of highly susceptible prion protein genotype VRQ/VRQ introduced into this decontaminated barn was carried out in addition to sampling and analysis of dust samples that were collected during the bioassay. Twenty‐four of the animals examined by immunohistochemical analysis of lymphatic tissues were scrapie‐positive during the bioassay, samples of dust collected within the barn were positive by month 3. The data illustrates the difficulty in decontaminating farm buildings from scrapie, and demonstrates the likely contribution of farm dust to the recontamination of these environments to levels that are capable of causing disease.

snip...

This study clearly demonstrates the difficulty in removing scrapie infectivity from the farm environment. Practical and effective prion decontamination methods are still urgently required for decontamination of scrapie infectivity from farms that have had cases of scrapie and this is particularly relevant for scrapiepositive goatherds, which currently have limited genetic resistance to scrapie within commercial breeds.24 This is very likely to have parallels with control efforts for CWD in cervids.

https://bvajournals.onlinelibrary.wiley.com/doi/abs/10.1136/vr.105054

***>This is very likely to have parallels with control efforts for CWD in cervids.

http://scrapie-usa.blogspot.com/2018/04/scrapie-transmits-to-pigs-by-oral-route.html

http://tseac.blogspot.com/2011/02/usa-50-state-bse-mad-cow-conference.html

https://bvajournals.onlinelibrary.wiley.com/doi/abs/10.1136/vr.105054

https://insights.ovid.com/veterinary-record/tvre/2019/01/190/rapid-recontamination-farm-building-occurs/19/00008049

https://search.proquest.com/openview/4544d5837142a98dd1bc8c1e32e79984/1?pq-origsite=gscholar&cbl=2041027

https://pubmed.ncbi.nlm.nih.gov/30602491/

***> Infectious agent of sheep scrapie may persist in the environment for at least 16 years

***> Nine of these recurrences occurred 14–21 years after culling, apparently as the result of environmental contamination, but outside entry could not always be absolutely excluded.

JOURNAL OF GENERAL VIROLOGY Volume 87, Issue 12

Infectious agent of sheep scrapie may persist in the environment for at least 16 years Free

Gudmundur Georgsson1, Sigurdur Sigurdarson2, Paul Brown3

http://www.microbiologyresearch.org/docserver/fulltext/jgv/87/12/3737.pdf?expires=1540908280&id=id&accname=guest&checksum=ED0572E1E5B272C100A32212A3E3761A

Saturday, January 5, 2019

Rapid recontamination of a farm building occurs after attempted prion removal

https://prionprp.blogspot.com/2019/01/rapid-recontamination-of-farm-building.html

The effectiveness of on-farm decontamination methods for scrapie - SE1865

Description

Scrapie infectivity persists on farms where infected animals have been removed1. Recently we have demonstrated that it is possible to detect environmental scrapie contamination biochemically using serial Protein Misfolding Cyclic Amplification (sPMCA)2, allowing the monitoring of scrapie infectivity on farm premises. Ongoing Defra study SE1863 has compared pen decontamination regimes on a scrapie-infected farm by both sheep bioassay and sPMCA. For bioassay, scrapie-free genetically susceptible lambs were introduced into pens decontaminated using distinct methodologies, all pens contained scrapie-positive lambs within 1 year. Remarkably this included lambs housed within a pen which had been jet washed/chloros treated, followed by regalvanisation/ replacement of all metalwork and painting of all other surfaces.

We have recently demonstrated using sPMCA, that material collected on swabs from vertical surfaces at heights inaccessible to sheep within a barn on the same scrapie affected farm contained scrapie prions (unpublished observations). We hypothesise that scrapie prions are most likely to have been deposited in these areas by bioaerosol movement. We propose that this bioaerosol movement contributes to scrapie transmission within the barn, and could account for the sheep that became positive within the pen containing re-galvanised/new metalwork and repainted surfaces (project SE1863). It is proposed that a thorough decontamination that would minimise prion-contaminated dust, both within the building and its immediate vicinity, is likely to increase the effectiveness of current methods for decontaminating farm buildings following outbreaks of scrapie. The proposed study builds on our previous data and will thoroughly investigate the potential for farm building scrapie-contamination via the bioaerosol route, a previously unrecognised route for dissemination of scrapie infectivity. This route could lead to the direct infection of healthy animals and/or indirect transmission of disease via contamination of surfaces within animal pens. The proposed study would analyse material collected using air samplers set up within “scrapie-infected” barns and their immediate vicinity, to confirm that prion containing material can be airborne within a scrapie infected farm environment. The study would incorporate a biochemical assessment of different surface decontamination methods, in order to demonstrate the best methodology and then the analysis of air and surface samples after a complete building decontamination to remove sources of dust and surface bound prions from both the building and its immediate vicinity. Analysis of such surface and air samples collected before and after treatment would measure the reduction in levels of infectivity. It is envisaged that the biochemical demonstration of airborne prions and the effective reduction in such prion dissemination would lead to a sheep bioassay experiment that would be conducted after a full farm decontamination. This would fully assess the effectiveness of an optimised scrapie decontamination strategy.

This study will contribute directly to Defra policy on best practice for on-farm decontamination after outbreaks of scrapie; a situation particularly relevant to decontamination after scrapie cases on goat farms where no genetic resistance to scrapie has currently been identified, and where complete decontamination is essential in order to stop recurrence of scrapie after restocking.

Objective

Phase 1

• Determine the presence and relative levels of airborne prions on a scrapie infected farm.

• Evaluate different pen surface decontamination procedures.

Phase 2

• Determine the presence of any airborne prions in a barn after a full decontamination.

Phase 3

• Further assess the efficacy of the decontamination procedure investigated in phase 2 by sheep bioassay.

Time-Scale and Cost

From: 2012

To: 2016

Cost: £326,784

Contractor / Funded Organisations

A D A S UK Ltd (ADAS)

Keywords Animals Fields of Study Animal Health

http://randd.defra.gov.uk/Default.aspx?FromSearch=Y&Location=None&Menu=Menu&Module=More&Paging=10&ProjectID=18479&Publisher=1&SearchText=SE1865&SortOrder=Asc&SortString=ProjectCode#Description

The Effectiveness of on-Farm Decontamination Methods for Scrapie

Institutions ADAS

Start date 2012

End date 2016

Objective Phase 1

Determine the presence and relative levels of airborne prions on a scrapie infected farm. Evaluate different pen surface decontamination procedures.

Phase 2

Determine the presence of any airborne prions in a barn after a full decontamination.

Phase 3

Further assess the efficacy of the decontamination procedure investigated in phase 2 by sheep bioassay.

More information

Scrapie infectivity persists on farms where infected animals have been removed1. Recently we have demonstrated that it is possible to detect environmental scrapie contamination biochemically using serial Protein Misfolding Cyclic Amplification (sPMCA)2, allowing the monitoring of scrapie infectivity on farm premises. Ongoing Defra study SE1863 has compared pen decontamination regimes on a scrapie-infected farm by both sheep bioassay and sPMCA. For bioassay, scrapie-free genetically susceptible lambs were introduced into pens decontaminated using distinct methodologies, all pens contained scrapie-positive lambs within 1 year. Remarkably this included lambs housed within a pen which had been jet washed/chloros treated, followed by regalvanisation/replacement of all metalwork and painting of all other surfaces.

We have recently demonstrated using sPMCA, that material collected on swabs from vertical surfaces at heights inaccessible to sheep within a barn on the same scrapie affected farm contained scrapie prions (unpublished observations). We hypothesise that scrapie prions are most likely to have been deposited in these areas by bioaerosol movement. We propose that this bioaerosol movement contributes to scrapie transmission within the barn, and could account for the sheep that became positive within the pen containing re-galvanised/new metalwork and repainted surfaces (project SE1863). It is proposed that a thorough decontamination that would minimise prion-contaminated dust, both within the building and its immediate vicinity, is likely to increase the effectiveness of current methods for decontaminating farm buildings following outbreaks of scrapie. The proposed study builds on our previous data and will thoroughly investigate the potential for farm building scrapie contamination via the bioaerosol route, a previously unrecognised route for dissemination of scrapie infectivity. This route could lead to the direct infection of healthy animals and/or indirect transmission of disease via contamination of surfaces within animal pens. The proposed study would analyse material collected using air samplers set up within “scrapie-infected” barns and their immediate vicinity, to confirm that prion containing material can be airborne within a scrapie infected farm environment. The study would incorporate a biochemical assessment of different surface decontamination methods, in order to demonstrate the best methodology and then the analysis of air and surface samples after a complete building decontamination to remove sources of dust and surface bound prions from both the building and its immediate vicinity. Analysis of such surface and air samples collected before and after treatment would measure the reduction in levels of infectivity. It is envisaged that the biochemical demonstration of airborne prions and the effective reduction in such prion dissemination would lead to a sheep bioassay experiment that would be conducted after a full farm decontamination. This would fully assess the effectiveness of an optimised scrapie decontamination strategy.

Funding Source

Department for Environment, Food and Rural Affairs

Project source

View this project

Project number

SE1865

Categories

Foodborne Disease

Policy and Planning

https://fsrio.nal.usda.gov/fsrio/research-projects/effectiveness-farm-decontamination-methods-scrapie

Circulation of prions within dust on a scrapie affected farm

Kevin C Gough1 , Claire A Baker2 , Hugh A Simmons3 , Steve A Hawkins3 and Ben C Maddison2*

Abstract

Prion diseases are fatal neurological disorders that affect humans and animals. Scrapie of sheep/goats and Chronic Wasting Disease (CWD) of deer/elk are contagious prion diseases where environmental reservoirs have a direct link to the transmission of disease. Using protein misfolding cyclic amplification we demonstrate that scrapie PrPSc can be detected within circulating dusts that are present on a farm that is naturally contaminated with sheep scrapie. The presence of infectious scrapie within airborne dusts may represent a possible route of infection and illustrates the difficulties that may be associated with the effective decontamination of such scrapie affected premises.

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Discussion We present biochemical data illustrating the airborne movement of scrapie containing material within a contaminated farm environment. We were able to detect scrapie PrPSc within extracts from dusts collected over a 70 day period, in the absence of any sheep activity. We were also able to detect scrapie PrPSc within dusts collected within pasture at 30 m but not at 60 m distance away from the scrapie contaminated buildings, suggesting that the chance of contamination of pasture by scrapie contaminated dusts decreases with distance from contaminated farm buildings. PrPSc amplification by sPMCA has been shown to correlate with infectivity and amplified products have been shown to be infectious [14,15]. These experiments illustrate the potential for low dose scrapie infectivity to be present within such samples. We estimate low ng levels of scrapie positive brain equivalent were deposited per m2 over 70 days, in a barn previously occupied by sheep affected with scrapie. This movement of dusts and the accumulation of low levels of scrapie infectivity within this environment may in part explain previous observations where despite stringent pen decontamination regimens healthy lambs still became scrapie infected after apparent exposure from their environment alone [16]. The presence of sPMCA seeding activity and by inference, infectious prions within dusts, and their potential for airborne dissemination is highly novel and may have implications for the spread of scrapie within infected premises. The low level circulation and accumulation of scrapie prion containing dust material within the farm environment will likely impede the efficient decontamination of such scrapie contaminated buildings unless all possible reservoirs of dust are removed. Scrapie containing dusts could possibly infect animals during feeding and drinking, and respiratory and conjunctival routes may also be involved. It has been demonstrated that scrapie can be efficiently transmitted via the nasal route in sheep [17], as is also the case for CWD in both murine models and in white tailed deer [18-20].

The sources of dust borne prions are unknown but it seems reasonable to assume that faecal, urine, skin, parturient material and saliva-derived prions may contribute to this mobile environmental reservoir of infectivity. This work highlights a possible transmission route for scrapie within the farm environment, and this is likely to be paralleled in CWD which shows strong similarities with scrapie in terms of prion dissemination and disease transmission. The data indicate that the presence of scrapie prions in dust is likely to make the control of these diseases a considerable challenge.

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4397813/

Research Project: TRANSMISSION, DIFFERENTIATION, AND PATHOBIOLOGY OF TRANSMISSIBLE SPONGIFORM ENCEPHALOPATHIES Location: Virus and Prion Research

Title: Scrapie transmits to white-tailed deer by the oral route and has a molecular profile similar to chronic wasting disease

Author

item Greenlee, Justin item Moore, S - Orise Fellow item Smith, Jodi - Iowa State University item Kunkle, Robert item West Greenlee, M - Iowa State University Submitted to: American College of Veterinary Pathologists Meeting Publication Type: Abstract Only Publication Acceptance Date: 8/12/2015 Publication Date: N/A Citation: N/A

Interpretive Summary:

Technical Abstract: The purpose of this work was to determine susceptibility of white-tailed deer (WTD) to the agent of sheep scrapie and to compare the resultant PrPSc to that of the original inoculum and chronic wasting disease (CWD). We inoculated WTD by a natural route of exposure (concurrent oral and intranasal (IN); n=5) with a US scrapie isolate. All scrapie-inoculated deer had evidence of PrPSc accumulation. PrPSc was detected in lymphoid tissues at preclinical time points, and deer necropsied after 28 months post-inoculation had clinical signs, spongiform encephalopathy, and widespread distribution of PrPSc in neural and lymphoid tissues. Western blotting (WB) revealed PrPSc with 2 distinct molecular profiles. WB on cerebral cortex had a profile similar to the original scrapie inoculum, whereas WB of brainstem, cerebellum, or lymph nodes revealed PrPSc with a higher profile resembling CWD. Homogenates with the 2 distinct profiles from WTD with clinical scrapie were further passaged to mice expressing cervid prion protein and intranasally to sheep and WTD. In cervidized mice, the two inocula have distinct incubation times. Sheep inoculated intranasally with WTD derived scrapie developed disease, but only after inoculation with the inoculum that had a scrapie-like profile. The WTD study is ongoing, but deer in both inoculation groups are positive for PrPSc by rectal mucosal biopsy. In summary, this work demonstrates that WTD are susceptible to the agent of scrapie, two distinct molecular profiles of PrPSc are present in the tissues of affected deer, and inoculum of either profile readily passes to deer.

https://www.ars.usda.gov/research/publications/publication/?seqNo115=317901

THE tse prion aka mad cow type disease is not your normal pathogen.

The TSE prion disease survives ashing to 600 degrees celsius, that’s around 1112 degrees farenheit.

you cannot cook the TSE prion disease out of meat.

you can take the ash and mix it with saline and inject that ash into a mouse, and the mouse will go down with TSE.

Prion Infected Meat-and-Bone Meal Is Still Infectious after Biodiesel Production as well.

the TSE prion agent also survives Simulated Wastewater Treatment Processes.

IN fact, you should also know that the TSE Prion agent will survive in the environment for years, if not decades.

you can bury it and it will not go away.

The TSE agent is capable of infected your water table i.e. Detection of protease-resistant cervid prion protein in water from a CWD-endemic area.

it’s not your ordinary pathogen you can just cook it out and be done with.

***> that’s what’s so worrisome about Iatrogenic mode of transmission, a simple autoclave will not kill this TSE prion agent.

1: J Neurol Neurosurg Psychiatry 1994 Jun;57(6):757-8

***> Transmission of Creutzfeldt-Jakob disease to a chimpanzee by electrodes contaminated during neurosurgery.

Gibbs CJ Jr, Asher DM, Kobrine A, Amyx HL, Sulima MP, Gajdusek DC.

Laboratory of Central Nervous System Studies, National Institute of

Neurological Disorders and Stroke, National Institutes of Health,

Bethesda, MD 20892.

Stereotactic multicontact electrodes used to probe the cerebral cortex of a middle aged woman with progressive dementia were previously implicated in the accidental transmission of Creutzfeldt-Jakob disease (CJD) to two younger patients. The diagnoses of CJD have been confirmed for all three cases. More than two years after their last use in humans, after three cleanings and repeated sterilisation in ethanol and formaldehyde vapour, the electrodes were implanted in the cortex of a chimpanzee. Eighteen months later the animal became ill with CJD. This finding serves to re-emphasise the potential danger posed by reuse of instruments contaminated with the agents of spongiform encephalopathies, even after scrupulous attempts to clean them.

PMID: 8006664 [PubMed - indexed for MEDLINE]

https://www.ncbi.nlm.nih.gov/pubmed/8006664?dopt=Abstract

New studies on the heat resistance of hamster-adapted scrapie agent: Threshold survival after ashing at 600°C suggests an inorganic template of replication

http://www.pnas.org/content/97/7/3418.full

Prion Infected Meat-and-Bone Meal Is Still Infectious after Biodiesel Production

http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2493038/

MONDAY, APRIL 19, 2021

Evaluation of the application for new alternative biodiesel production process for rendered fat including Category 1 animal by-products (BDI-RepCat® process, AT) ???

https://transmissiblespongiformencephalopathy.blogspot.com/2021/04/evaluation-of-application-for-new.html

Detection of protease-resistant cervid prion protein in water from a CWD-endemic area

https://www.ncbi...nlm.nih.gov/pmc/articles/PMC2802782/pdf/prion0303_0171.pdf

A Quantitative Assessment of the Amount of Prion Diverted to Category 1 Materials and Wastewater During Processing

http://onlinelibrary.wiley.com/doi/10.1111/j.1539-6924.2012.01922.x/abstract

Rapid assessment of bovine spongiform encephalopathy prion inactivation by heat treatment in yellow grease produced in the industrial manufacturing process of meat and bone meals

https://bmcvetres.biomedcentral.com/track/pdf/10.1186/1746-6148-9-134.pdf

THURSDAY, FEBRUARY 28, 2019

BSE infectivity survives burial for five years with only limited spread

https://link.springer.com/content/pdf/10.1007%2Fs00705-019-04154-8.pdf

***> CONGRESSIONAL ABSTRACTS PRION CONFERENCE 2018

P69 Experimental transmission of CWD from white-tailed deer to co-housed reindeer

Mitchell G (1), Walther I (1), Staskevicius A (1), Soutyrine A (1), Balachandran A (1)

(1) National & OIE Reference Laboratory for Scrapie and CWD, Canadian Food Inspection Agency, Ottawa, Ontario, Canada.

Chronic wasting disease (CWD) continues to be detected in wild and farmed cervid populations of North America, affecting predominantly white-tailed deer, mule deer and elk. Extensive herds of wild caribou exist in northern regions of Canada, although surveillance has not detected the presence of CWD in this population. Oral experimental transmission has demonstrated that reindeer, a species closely related to caribou, are susceptible to CWD. Recently, CWD was detected for the first time in Europe, in wild Norwegian reindeer, advancing the possibility that caribou in North America could also become infected. Given the potential overlap in habitat between wild CWD-infected cervids and wild caribou herds in Canada, we sought to investigate the horizontal transmissibility of CWD from white-tailed deer to reindeer.

Two white-tailed deer were orally inoculated with a brain homogenate prepared from a farmed Canadian white-tailed deer previously diagnosed with CWD. Two reindeer, with no history of exposure to CWD, were housed in the same enclosure as the white-tailed deer, 3.5 months after the deer were orally inoculated. The white-tailed deer developed clinical signs consistent with CWD beginning at 15.2 and 21 months post-inoculation (mpi), and were euthanized at 18.7 and 23.1 mpi, respectively. Confirmatory testing by immunohistochemistry (IHC) and western blot demonstrated widespread aggregates of pathological prion protein (PrPCWD) in the central nervous system and lymphoid tissues of both inoculated white-tailed deer. Both reindeer were subjected to recto-anal mucosal associated lymphoid tissue (RAMALT) biopsy at 20 months post-exposure (mpe) to the white-tailed deer. The biopsy from one reindeer contained PrPCWD confirmed by IHC. This reindeer displayed only subtle clinical evidence of disease prior to a rapid decline in condition requiring euthanasia at 22.5 mpe. Analysis of tissues from this reindeer by IHC revealed widespread PrPCWD deposition, predominantly in central nervous system and lymphoreticular tissues. Western blot molecular profiles were similar between both orally inoculated white-tailed deer and the CWD positive reindeer. Despite sharing the same enclosure, the other reindeer was RAMALT negative at 20 mpe, and PrPCWD was not detected in brainstem and lymphoid tissues following necropsy at 35 mpe. Sequencing of the prion protein gene from both reindeer revealed differences at several codons, which may have influenced susceptibility to infection.

Natural transmission of CWD occurs relatively efficiently amongst cervids, supporting the expanding geographic distribution of disease and the potential for transmission to previously naive populations. The efficient horizontal transmission of CWD from white-tailed deer to reindeer observed here highlights the potential for reindeer to become infected if exposed to other cervids or environments infected with CWD.

SOURCE REFERENCE 2018 PRION CONFERENCE ABSTRACT

Research Project: TRANSMISSION, DIFFERENTIATION, AND PATHOBIOLOGY OF TRANSMISSIBLE SPONGIFORM ENCEPHALOPATHIES Location: Virus and Prion Research

Title: Horizontal transmission of chronic wasting disease in reindeer

Author

item MOORE, SARAH - ORISE FELLOW item KUNKLE, ROBERT item WEST GREENLEE, MARY - IOWA STATE UNIVERSITY item Nicholson, Eric item RICHT, JUERGEN item HAMIR, AMIRALI item WATERS, WADE item Greenlee, Justin

Submitted to: Emerging Infectious Diseases

Publication Type: Peer Reviewed Journal

Publication Acceptance Date: 8/29/2016

Publication Date: 12/1/2016

Citation: Moore, S., Kunkle, R., Greenlee, M., Nicholson, E., Richt, J., Hamir, A., Waters, W., Greenlee, J. 2016. Horizontal transmission of chronic wasting disease in reindeer. Emerging Infectious Diseases. 22(12):2142-2145. doi:10.3201/eid2212.160635.

Interpretive Summary: Chronic wasting disease (CWD) is a fatal neurodegenerative disease that occurs in farmed and wild cervids (deer and elk) of North America and was recently diagnosed in a single free-ranging reindeer (Rangifer tarandus tarandus) in Norway. CWD is a transmissible spongiform encephalopathy (TSE) that is caused by infectious proteins called prions that are resistant to various methods of decontamination and environmental degradation. Little is known about the susceptibility of or potential for transmission amongst reindeer. In this experiment, we tested the susceptibility of reindeer to CWD from various sources (elk, mule deer, or white-tailed deer) after intracranial inoculation and tested the potential for infected reindeer to transmit to non-inoculated animals by co-housing or housing in adjacent pens. Reindeer were susceptible to CWD from elk, mule deer, or white-tailed deer sources after experimental inoculation. Most importantly, non-inoculated reindeer that were co-housed with infected reindeer or housed in pens adjacent to infected reindeer but without the potential for nose-to-nose contact also developed evidence of CWD infection. This is a major new finding that may have a great impact on the recently diagnosed case of CWD in the only remaining free-ranging reindeer population in Europe as our findings imply that horizontal transmission to other reindeer within that herd has already occurred. Further, this information will help regulatory and wildlife officials developing plans to reduce or eliminate CWD and cervid farmers that want to ensure that their herd remains CWD-free, but were previously unsure of the potential for reindeer to transmit CWD.

Technical Abstract: Chronic wasting disease (CWD) is a naturally-occurring, fatal prion disease of cervids. Reindeer (Rangifer tarandus tarandus) are susceptible to CWD following oral challenge, and CWD was recently reported in a free-ranging reindeer of Norway. Potential contact between CWD-affected cervids and Rangifer species that are free-ranging or co-housed on farms presents a potential risk of CWD transmission. The aims of this study were to 1) investigate the transmission of CWD from white-tailed deer (Odocoileus virginianus; CWDwtd), mule deer (Odocoileus hemionus; CWDmd), or elk (Cervus elaphus nelsoni; CWDelk) to reindeer via the intracranial route, and 2) to assess for direct and indirect horizontal transmission to non-inoculated sentinels. Three groups of 5 reindeer fawns were challenged intracranially with CWDwtd, CWDmd, or CWDelk. Two years after challenge of inoculated reindeer, non-inoculated negative control reindeer were introduced into the same pen as the CWDwtd inoculated reindeer (direct contact; n=4) or into a pen adjacent to the CWDmd inoculated reindeer (indirect contact; n=2). Experimentally inoculated reindeer were allowed to develop clinical disease. At death/euthanasia a complete necropsy examination was performed, including immunohistochemical testing of tissues for disease-associated CWD prion protein (PrPcwd). Intracranially challenged reindeer developed clinical disease from 21 months post-inoculation (months PI). PrPcwd was detected in 5 out of 6 sentinel reindeer although only 2 out of 6 developed clinical disease during the study period (< 57 months PI). We have shown that reindeer are susceptible to CWD from various cervid sources and can transmit CWD to naïve reindeer both directly and indirectly.

https://www.ars.usda.gov/research/publications/publication/?seqNo115=328261

Infectivity surviving ashing to 600*C is (in my opinion) degradable but infective. based on Bown & Gajdusek, (1991), landfill and burial may be assumed to have a reduction factor of 98% (i.e. a factor of 50) over 3 years. CJD-infected brain-tissue remained infectious after storing at room-temperature for 22 months (Tateishi et al, 1988). Scrapie agent is known to remain viable after at least 30 months of desiccation (Wilson et al, 1950). and pastures that had been grazed by scrapie-infected sheep still appeared to be contaminated with scrapie agent three years after they were last occupied by sheep (Palsson, 1979).

http://europa.eu.int/comm/food/fs/sc/ssc/out58_en.pdf

Dr. Paul Brown Scrapie Soil Test BSE Inquiry Document

https://web.archive.org/web/20090505211734/http://www.bseinquiry.gov.uk/files/sc/Seac07/tab03.pdf

Using in vitro Prion replication for high sensitive detection of prions and prionlike proteins and for understanding mechanisms of transmission.

Claudio Soto Mitchell Center for Alzheimer's diseases and related Brain disorders, Department of Neurology, University of Texas Medical School at Houston.

Prion and prion-like proteins are misfolded protein aggregates with the ability to selfpropagate to spread disease between cells, organs and in some cases across individuals. I n T r a n s m i s s i b l e s p o n g i f o r m encephalopathies (TSEs), prions are mostly composed by a misfolded form of the prion protein (PrPSc), which propagates by transmitting its misfolding to the normal prion protein (PrPC). The availability of a procedure to replicate prions in the laboratory may be important to study the mechanism of prion and prion-like spreading and to develop high sensitive detection of small quantities of misfolded proteins in biological fluids, tissues and environmental samples. Protein Misfolding Cyclic Amplification (PMCA) is a simple, fast and efficient methodology to mimic prion replication in the test tube. PMCA is a platform technology that may enable amplification of any prion-like misfolded protein aggregating through a seeding/nucleation process. In TSEs, PMCA is able to detect the equivalent of one single molecule of infectious PrPSc and propagate prions that maintain high infectivity, strain properties and species specificity. Using PMCA we have been able to detect PrPSc in blood and urine of experimentally infected animals and humans affected by vCJD with high sensitivity and specificity. Recently, we have expanded the principles of PMCA to amplify amyloid-beta (Aβ) and alphasynuclein (α-syn) aggregates implicated in Alzheimer's and Parkinson's diseases, respectively. Experiments are ongoing to study the utility of this technology to detect Aβ and α-syn aggregates in samples of CSF and blood from patients affected by these diseases.

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***>>> Recently, we have been using PMCA to study the role of environmental prion contamination on the horizontal spreading of TSEs. These experiments have focused on the study of the interaction of prions with plants and environmentally relevant surfaces. Our results show that plants (both leaves and roots) bind tightly to prions present in brain extracts and excreta (urine and feces) and retain even small quantities of PrPSc for long periods of time. Strikingly, ingestion of prioncontaminated leaves and roots produced disease with a 100% attack rate and an incubation period not substantially longer than feeding animals directly with scrapie brain homogenate. Furthermore, plants can uptake prions from contaminated soil and transport them to different parts of the plant tissue (stem and leaves). Similarly, prions bind tightly to a variety of environmentally relevant surfaces, including stones, wood, metals, plastic, glass, cement, etc. Prion contaminated surfaces efficiently transmit prion disease when these materials were directly injected into the brain of animals and strikingly when the contaminated surfaces were just placed in the animal cage. These findings demonstrate that environmental materials can efficiently bind infectious prions and act as carriers of infectivity, suggesting that they may play an important role in the horizontal transmission of the disease.

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Since its invention 13 years ago, PMCA has helped to answer fundamental questions of prion propagation and has broad applications in research areas including the food industry, blood bank safety and human and veterinary disease diagnosis.

source reference Prion Conference 2015 abstract book

Grass Plants Bind, Retain, Uptake, and Transport Infectious Prions

Sandra Pritzkow,1 Rodrigo Morales,1 Fabio Moda,1,3 Uffaf Khan,1 Glenn C. Telling,2 Edward Hoover,2 and Claudio Soto1, * 1Mitchell Center for Alzheimer’s Disease and Related Brain Disorders, Department of Neurology, University of Texas Medical School at Houston, 6431 Fannin Street, Houston, TX 77030, USA

2Prion Research Center, Department of Microbiology, Immunology, and Pathology, College of Veterinary Medicine and Biomedical Sciences, Colorado State University, Fort Collins, CO 80523, USA

3Present address: IRCCS Foundation Carlo Besta Neurological Institute, 20133 Milan, Italy *Correspondence: claudio.soto@uth.tmc.edu http://dx.doi.org/10.1016/j.celrep.2015.04.036

SUMMARY

Prions are the protein-based infectious agents responsible for prion diseases. Environmental prion contamination has been implicated in disease transmission. Here, we analyzed the binding and retention of infectious prion protein (PrPSc) to plants. Small quantities of PrPSc contained in diluted brain homogenate or in excretory materials (urine and feces) can bind to wheat grass roots and leaves. Wild-type hamsters were efficiently infected by ingestion of prion-contaminated plants. The prion-plant interaction occurs with prions from diverse origins, including chronic wasting disease. Furthermore, leaves contaminated by spraying with a prion-containing preparation retained PrPSc for several weeks in the living plant. Finally, plants can uptake prions from contaminated soil and transport them to aerial parts of the plant (stem and leaves). These findings demonstrate that plants can efficiently bind infectious prions and act as carriers of infectivity, suggesting a possible role of environmental prion contamination in the horizontal transmission of the disease.

INTRODUCTION

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DISCUSSION

This study shows that plants can efficiently bind prions contained in brain extracts from diverse prion infected animals, including CWD-affected cervids. PrPSc attached to leaves and roots from wheat grass plants remains capable of seeding prion replication in vitro. Surprisingly, the small quantity of PrPSc naturally excreted in urine and feces from sick hamster or cervids was enough to efficiently contaminate plant tissue. Indeed, our results suggest that the majority of excreted PrPSc is efficiently captured by plants’ leaves and roots. Moreover, leaves can be contaminated by spraying them with a prion-containing extract, and PrPSc remains detectable in living plants for as long as the study was performed (several weeks). Remarkably, prion contaminated plants transmit prion disease to animals upon ingestion, producing a 100% attack rate and incubation periods not substantially longer than direct oral administration of sick brain homogenates.

Finally, an unexpected but exciting result was that plants were able to uptake prions from contaminated soil and transport them to aerial parts of the plant tissue. Although it may seem farfetched that plants can uptake proteins from the soil and transport it to the parts above the ground, there are already published reports of this phenomenon (McLaren et al., 1960; Jensen and McLaren, 1960;Paungfoo-Lonhienne et al., 2008). The high resistance of prions to degradation and their ability to efficiently cross biological barriers may play a role in this process. The mechanism by which plants bind, retain, uptake, and transport prions is unknown. We are currently studying the way in which prions interact with plants using purified, radioactively labeled PrPSc to determine specificity of the interaction, association constant, reversibility, saturation, movement, etc.

Epidemiological studies have shown numerous instances of scrapie or CWD recurrence upon reintroduction of animals on pastures previously exposed to prion-infected animals. Indeed, reappearance of scrapie has been documented following fallow periods of up to 16 years (Georgsson et al., 2006), and pastures were shown to retain infectious CWD prions for at least 2 years after exposure (Miller et al., 2004). It is likely that the environmentally mediated transmission of prion diseases depends upon the interaction of prions with diverse elements, including soil, water, environmental surfaces, various invertebrate animals, and plants.

However, since plants are such an important component of the environment and also a major source of food for many animal species, including humans, our results may have far-reaching implications for animal and human health. Currently, the perception of the riskfor animal-to-human prion transmission has beenmostly limited to consumption or exposure to contaminated meat; our results indicate that plants might also be an important vector of transmission that needs to be considered in risk assessment.

https://www.cell.com/cell-reports/pdf/S2211-1247%2815%2900437-4.pdf

RIGINAL RESEARCH ARTICLE

Front. Vet. Sci., 14 September 2015 | https://doi.org/10.3389/fvets.2015.00032

Objects in contact with classical scrapie sheep act as a reservoir for scrapie transmission

imageTimm Konold1*, imageStephen A. C. Hawkins2, imageLisa C. Thurston3, imageBen C. Maddison4, imageKevin C. Gough5, imageAnthony Duarte1 and imageHugh A. Simmons1

1Animal Sciences Unit, Animal and Plant Health Agency Weybridge, Addlestone, UK

2Pathology Department, Animal and Plant Health Agency Weybridge, Addlestone, UK

3Surveillance and Laboratory Services, Animal and Plant Health Agency Penrith, Penrith, UK

4ADAS UK, School of Veterinary Medicine and Science, University of Nottingham, Sutton Bonington, UK

5School of Veterinary Medicine and Science, University of Nottingham, Sutton Bonington, UK

Classical scrapie is an environmentally transmissible prion disease of sheep and goats. Prions can persist and remain potentially infectious in the environment for many years and thus pose a risk of infecting animals after re-stocking. In vitro studies using serial protein misfolding cyclic amplification (sPMCA) have suggested that objects on a scrapie-affected sheep farm could contribute to disease transmission. This in vivo study aimed to determine the role of field furniture (water troughs, feeding troughs, fencing, and other objects that sheep may rub against) used by a scrapie-infected sheep flock as a vector for disease transmission to scrapie-free lambs with the prion protein genotype VRQ/VRQ, which is associated with high susceptibility to classical scrapie. When the field furniture was placed in clean accommodation, sheep became infected when exposed to either a water trough (four out of five) or to objects used for rubbing (four out of seven). This field furniture had been used by the scrapie-infected flock 8 weeks earlier and had previously been shown to harbor scrapie prions by sPMCA. Sheep also became infected (20 out of 23) through exposure to contaminated field furniture placed within pasture not used by scrapie-infected sheep for 40 months, even though swabs from this furniture tested negative by PMCA. This infection rate decreased (1 out of 12) on the same paddock after replacement with clean field furniture. Twelve grazing sheep exposed to field furniture not in contact with scrapie-infected sheep for 18 months remained scrapie free. The findings of this study highlight the role of field furniture used by scrapie-infected sheep to act as a reservoir for disease re-introduction although infectivity declines considerably if the field furniture has not been in contact with scrapie-infected sheep for several months. PMCA may not be as sensitive as VRQ/VRQ sheep to test for environmental contamination.

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Discussion

Classical scrapie is an environmentally transmissible disease because it has been reported in naïve, supposedly previously unexposed sheep placed in pastures formerly occupied by scrapie-infected sheep (4, 19, 20).

Although the vector for disease transmission is not known, soil is likely to be an important reservoir for prions (2) where – based on studies in rodents – prions can adhere to minerals as a biologically active form (21) and remain infectious for more than 2 years (22).

Similarly, chronic wasting disease (CWD) has re-occurred in mule deer housed in paddocks used by infected deer 2 years earlier, which was assumed to be through foraging and soil consumption (23).

Our study suggested that the risk of acquiring scrapie infection was greater through exposure to contaminated wooden, plastic, and metal surfaces via water or food troughs, fencing, and hurdles than through grazing.

Drinking from a water trough used by the scrapie flock was sufficient to cause infection in sheep in a clean building.

Exposure to fences and other objects used for rubbing also led to infection, which supported the hypothesis that skin may be a vector for disease transmission (9).

The risk of these objects to cause infection was further demonstrated when 87% of 23 sheep presented with PrPSc in lymphoid tissue after grazing on one of the paddocks, which contained metal hurdles, a metal lamb creep and a water trough in contact with the scrapie flock up to 8 weeks earlier, whereas no infection had been demonstrated previously in sheep grazing on this paddock, when equipped with new fencing and field furniture.

When the contaminated furniture and fencing were removed, the infection rate dropped significantly to 8% of 12 sheep, with soil of the paddock as the most likely source of infection caused by shedding of prions from the scrapie-infected sheep in this paddock up to a week earlier.

This study also indicated that the level of contamination of field furniture sufficient to cause infection was dependent on two factors: stage of incubation period and time of last use by scrapie-infected sheep.

Drinking from a water trough that had been used by scrapie sheep in the predominantly pre-clinical phase did not appear to cause infection, whereas infection was shown in sheep drinking from the water trough used by scrapie sheep in the later stage of the disease.

It is possible that contamination occurred through shedding of prions in saliva, which may have contaminated the surface of the water trough and subsequently the water when it was refilled.

Contamination appeared to be sufficient to cause infection only if the trough was in contact with sheep that included clinical cases.

Indeed, there is an increased risk of bodily fluid infectivity with disease progression in scrapie (24) and CWD (25) based on PrPSc detection by sPMCA.

Although ultraviolet light and heat under natural conditions do not inactivate prions (26), furniture in contact with the scrapie flock, which was assumed to be sufficiently contaminated to cause infection, did not act as vector for disease if not used for 18 months, which suggest that the weathering process alone was sufficient to inactivate prions.

PrPSc detection by sPMCA is increasingly used as a surrogate for infectivity measurements by bioassay in sheep or mice.

In this reported study, however, the levels of PrPSc present in the environment were below the limit of detection of the sPMCA method, yet were still sufficient to cause infection of in-contact animals.

In the present study, the outdoor objects were removed from the infected flock 8 weeks prior to sampling and were positive by sPMCA at very low levels (2 out of 37 reactions).

As this sPMCA assay also yielded 2 positive reactions out of 139 in samples from the scrapie-free farm, the sPMCA assay could not detect PrPSc on any of the objects above the background of the assay.

False positive reactions with sPMCA at a low frequency associated with de novo formation of infectious prions have been reported (27, 28).

This is in contrast to our previous study where we demonstrated that outdoor objects that had been in contact with the scrapie-infected flock up to 20 days prior to sampling harbored PrPSc that was detectable by sPMCA analysis [4 out of 15 reactions (12)] and was significantly more positive by the assay compared to analogous samples from the scrapie-free farm.

This discrepancy could be due to the use of a different sPMCA substrate between the studies that may alter the efficiency of amplification of the environmental PrPSc.

In addition, the present study had a longer timeframe between the objects being in contact with the infected flock and sampling, which may affect the levels of extractable PrPSc.

Alternatively, there may be potentially patchy contamination of this furniture with PrPSc, which may have been missed by swabbing.

The failure of sPMCA to detect CWD-associated PrP in saliva from clinically affected deer despite confirmation of infectivity in saliva-inoculated transgenic mice was associated with as yet unidentified inhibitors in saliva (29), and it is possible that the sensitivity of sPMCA is affected by other substances in the tested material.

In addition, sampling of amplifiable PrPSc and subsequent detection by sPMCA may be more difficult from furniture exposed to weather, which is supported by the observation that PrPSc was detected by sPMCA more frequently in indoor than outdoor furniture (12).

A recent experimental study has demonstrated that repeated cycles of drying and wetting of prion-contaminated soil, equivalent to what is expected under natural weathering conditions, could reduce PMCA amplification efficiency and extend the incubation period in hamsters inoculated with soil samples (30).

This seems to apply also to this study even though the reduction in infectivity was more dramatic in the sPMCA assays than in the sheep model.

Sheep were not kept until clinical end-point, which would have enabled us to compare incubation periods, but the lack of infection in sheep exposed to furniture that had not been in contact with scrapie sheep for a longer time period supports the hypothesis that prion degradation and subsequent loss of infectivity occurs even under natural conditions.

In conclusion, the results in the current study indicate that removal of furniture that had been in contact with scrapie-infected animals should be recommended, particularly since cleaning and decontamination may not effectively remove scrapie infectivity (31), even though infectivity declines considerably if the pasture and the field furniture have not been in contact with scrapie-infected sheep for several months. As sPMCA failed to detect PrPSc in furniture that was subjected to weathering, even though exposure led to infection in sheep, this method may not always be reliable in predicting the risk of scrapie infection through environmental contamination.

These results suggest that the VRQ/VRQ sheep model may be more sensitive than sPMCA for the detection of environmentally associated scrapie, and suggest that extremely low levels of scrapie contamination are able to cause infection in susceptible sheep genotypes.

Keywords: classical scrapie, prion, transmissible spongiform encephalopathy, sheep, field furniture, reservoir, serial protein misfolding cyclic amplification

http://journal.frontiersin.org/article/10.3389/fvets.2015.00032/full

EXOTIC SPECIES AND CWD TSE PRP

BSE TSE Prion in zoo animals, exotic ruminants, domestic cats, and CPD Camel Prion Disease, a review 2020

The BSE Inquiry / Statement No 324

Dr James Kirkwood

(not scheduled to give oral evidence)

Statement to the BSE Inquiry

http://web.archive.org/web/20090506004016/http://www.bseinquiry.gov.uk/files/ws/s324.pdf

TSEs in Exotic Ruminants

TSEs have been detected in exotic ruminants in UK zoos since 1986. These include antelopes (Eland, Gemsbok, Arabian and Scimitar oryx, Nyala and Kudu), Ankole cattle and Bison. With hindsight the 1986 case in a Nyala was diagnosed before the first case of BSE was identified. The TSE cases in exotic ruminants had a younger onset age and a shorter clinical duration compared to that in cattle with BSE. All the cases appear to be linked to the BSE epidemic via the consumption of feed contaminated with the BSE agent. The epidemic has declined as a result of tight controls on feeding mammalian meat and bone meal to susceptible animals, particularly from August 1996.

https://webarchive.nationalarchives.gov.uk/20130402160403/http://archive.defra.gov.uk/foodfarm/farmanimal/diseases/atoz/bse/othertses/index.htm#exotic

SPONGIFORM ENCEPHALOPATHY IN A CAPTIVE PUMA

an article in yesterday's Times (attached) which suggested that the puma concerned had never ''eaten any part of a cow or sheep which, in the opinion of Government Scientists, could transmit the species to a different species''.

3. You explained to me that this was INCORRECT. The position was as set out in the briefing for Prime Minister's questions attached to Mr Taylor's note. The puma had probably been fed low quality beef meat in the form of split carcasses. ...

http://web.archive.org/web/20090506032628/http://www.bseinquiry.gov.uk/files/yb/1992/11/13001001.pdf

Spongiform Encephalopathy in Captive Wild Animals in Britain

https://web.archive.org/web/20090506001201/http://www.bseinquiry.gov.uk/files/mb/m09a/tab03.pdf

Sun, Dec 20, 2020 4:54 pm

Subject: TSE in exotic ruminants

TSE in exotic ruminants

https://webarchive.nationalarchives.gov.uk/20130402160403/http://archive.defra.gov.uk/foodfarm/farmanimal/diseases/atoz/bse/othertses/index.htm#exotic

NUMBER OF CONFIRMED CASES OF FSE IN DOMESTIC CATS BY YEAR Year Reported No. of cases Year of Onset No. of cases

1988 0 1988 0

1989 0 1989 1

1990 12 1990 16

1991 12 1991 11

1992 10 1992 14

1993 11 1993 10

1994 16 1994 14

1995 8 1995 4

1996 6 1996 7

1997 6 1997 8

1998 4 1998 1

1999 2 1999 1

2000 1 2000 1

2001 1 2001 1

2002 0 2002 0

2003 0 2003 0

2004 0 2004 0

2005 0 2005 0

2006 0 2006 0

2007 0 2007 0

2008 0 2008 0

2009 0 2009 0

2010 0 2010 0

2011 0 2011 0

2012 0 2012 0

2013 0 2013 0

2014 0 2014 0

2015 0 2015 0

2016 0 2016 0

2017 0 2017 0

2018 0 2018 0

2019 0 2019 0

2020 0 2020 0

Total 89 Total 89 Data valid to 30 November 2020 Includes one case from Guernsey

Published 11 February 2015 Last updated 21 December 2020 - hide all updates

https://assets.publishing.service.gov.uk/government/uploads/system/uploads/attachment_data/file/946743/pub-tse-stats-exotic.ods

https://webarchive.nationalarchives.gov.uk/20130703015619/http://www.defra.gov.uk/ahvla-en/publication/tse-stats-exotic/?shared=email&msg=fail

https://www.gov.uk/government/collections/tse-disease-surveillance-statistics

https://www.gov.uk/government/publications/cattle-tse-surveillance-statistics

https://www.gov.uk/government/publications/active-tse-surveillance-statistics

http://caninespongiformencephalopathy.blogspot.com/2016/

SE DIAGNOSES IN EXOTIC SPECIES

KUDU 6

GEMSBOK 1

NYALA 1

ORYX 2

ELAND 6

CHEETAH 4*

PUMA 3

TIGER 1

OCELOT 2

BISON (bison bison) 1

ANKOLE COW 2

* Excludes one cheetah in Australia and one in ROI - litter mates born in GB, and another in France also born in G.B. [figures to 1 January 1998]

FELINE SPONGIFORM ENCEPHALOPATHY

TOTAL TO DATE 81 (Plus 1 in N Ireland, 1 in Norway, 1 in Lichtenstein )

YEAR Cases

1990 12

1991 12

1992 10

1993 11

1994 16

1995 8

1996 6

1997 6

https://webarchive.nationalarchives.gov.uk/19980215152423/http://www.maff.gov.uk:80/animalh/bse/bse-statistics/level-3-tsestat.html

Exotic species

Species Number of cases Dates affected

Ankole Cow 2 1991, 95

Bison 1 1996

Asian Leopard Cat (1) 1 2005

Cheetah 5 1992, 98

Eland 6 1989, 95

Gemsbok 1 1987

Kudu 6 1989, 92

Lion 4 1998, 2001

Nyala 1 1986

Ocelot 3 1994, 99

Oryx 2 1989, 92

Puma 3 1992, 95

Tiger 3 1995, 99

As at 12 January 2006.

A total of 38 cases of spongiform encephalopathy have been confirmed in exotic species, the last one in 2005.

(1) Felis (Prionailurus) bengalensis.

https://webarchive.nationalarchives.gov.uk/20060421120000/http://www.defra.gov.uk/animalh/bse/statistics/tsestats.html

BSE TSE PRION STATISTICS

https://webarchive.nationalarchives.gov.uk/19980215152404/http://www.maff.gov.uk:80/animalh/bse/bse-statistics/level-3-incidence.html

https://webarchive.nationalarchives.gov.uk/20130702132327/http://www.defra.gov.uk/ahvla-en/science/tse/surveillance-stats/

https://webarchive.nationalarchives.gov.uk/20141204110520/http://archive.defra.gov.uk/foodfarm/farmanimal/diseases/atoz/bse/othertses/index.htm

https://webarchive.nationalarchives.gov.uk/20080107221534/http://www.defra.gov.uk/animalh/bse/othertses/index.html

https://webarchive.nationalarchives.gov.uk/20150501025828/https://www.gov.uk/government/statistics/exotic-species-and-domestic-cats-tse-surveillance-statistics

https://webarchive.nationalarchives.gov.uk/search/result/?q=bse%20cheetah

ZOO ANIMALS AND TSE PRION DISEASE

The 82 zoo animals with BSE:

snip...

http://www.mad-cow.org/may99_zoo_news.html

http://www.mad-cow.org/99feb_cwd_special.html#fff

http://www.mad-cow.org/00/aug00_late_news.html#ggg

http://www.mad-cow.org/00/aug00_last_news.html#fff

http://www.pnas.org/content/96/7/4046.full

http://bseinquiry.blogspot.com/2008/05/bse-inquiry-draft-factual-account-dfa.html

http://felinespongiformencephalopathyfse.blogspot.com/2019/

2020

Monday, November 30, 2020

Tunisia has become the second country after Algeria to detect a case of CPD Camel Prion Disease within a year

REPORT OF THE MEETING OF THE OIE SCIENTIFIC COMMISSION FOR ANIMAL DISEASES Paris, 9–13 September 2019

Scientific Commission/September 2019

Tunisia has become the second country after Algeria to detect a case of CPD within a year

10.2. Prion disease in dromedary camels

https://camelusprp.blogspot.com/2020/11/

CWD AND SCRAPIE TRANSMIT TO PIGS BY ORAL ROUTES

Research Project: TRANSMISSION, DIFFERENTIATION, AND PATHOBIOLOGY OF TRANSMISSIBLE SPONGIFORM ENCEPHALOPATHIES Location: Virus and Prion Research

Title: Disease-associated prion protein detected in lymphoid tissues from pigs challenged with the agent of chronic wasting disease

Conclusions: This study demonstrates that PrPSc accumulates in lymphoid tissues from pigs challenged intracranially or orally with the CWD agent, and can be detected as early as 4 months after challenge. CWD-infected pigs rarely develop clinical disease and if they do, they do so after a long incubation period. This raises the possibility that CWD-infected pigs could shed prions into their environment long before they develop clinical disease. Furthermore, lymphoid tissues from CWD-infected pigs could present a potential source of CWD infectivity in the animal and human food chains.

https://www.ars.usda.gov/research/publications/publication/?seqNo115=337105

https://www.ars.usda.gov/research/publications/publication/?seqNo115=326166

Research Project: Pathobiology, Genetics, and Detection of Transmissible Spongiform Encephalopathies Location: Virus and Prion Research

Title: The agent of chronic wasting disease from pigs is infectious in transgenic mice expressing human PRNP

Author item MOORE, S - Orise Fellow item Kokemuller, Robyn item WEST-GREENLEE, M - Iowa State University item BALKEMA-BUSCHMANN, ANNE - Friedrich-Loeffler-institut item GROSCHUP, MARTIN - Friedrich-Loeffler-institut item Greenlee, Justin Submitted to: Prion Publication Type: Abstract Only Publication Acceptance Date: 5/10/2018 Publication Date: 5/22/2018 Citation: Moore, S.J., Kokemuller, R.D., West-Greenlee, M.H., Balkema-Buschmann, A., Groschup, M.H., Greenlee, J.J. 2018. The agent of chronic wasting disease from pigs is infectious in transgenic mice expressing human PRNP. Prion 2018, Santiago de Compostela, Spain, May 22-25, 2018. Paper No. WA15, page 44.

Interpretive Summary:

The successful transmission of pig-passaged CWD to Tg40 mice reported here suggests that passage of the CWD agent through pigs results in a change of the transmission characteristics which reduces the transmission barrier of Tg40 mice to the CWD agent. If this biological behavior is recapitulated in the original host species, passage of the CWD agent through pigs could potentially lead to increased pathogenicity of the CWD agent in humans.

https://www.ars.usda.gov/research/publications/publication/?seqNo115=353091

cwd scrapie pigs oral routes

***> However, at 51 months of incubation or greater, 5 animals were positive by one or more diagnostic methods. Furthermore, positive bioassay results were obtained from all inoculated groups (oral and intracranial; market weight and end of study) suggesting that swine are potential hosts for the agent of scrapie. <***

>*** Although the current U.S. feed ban is based on keeping tissues from TSE infected cattle from contaminating animal feed, swine rations in the U.S. could contain animal derived components including materials from scrapie infected sheep and goats. These results indicating the susceptibility of pigs to sheep scrapie, coupled with the limitations of the current feed ban, indicates that a revision of the feed ban may be necessary to protect swine production and potentially human health. <***

***> Results: PrPSc was not detected by EIA and IHC in any RPLNs. All tonsils and MLNs were negative by IHC, though the MLN from one pig in the oral <6 month group was positive by EIA. PrPSc was detected by QuIC in at least one of the lymphoid tissues examined in 5/6 pigs in the intracranial <6 months group, 6/7 intracranial >6 months group, 5/6 pigs in the oral <6 months group, and 4/6 oral >6 months group. Overall, the MLN was positive in 14/19 (74%) of samples examined, the RPLN in 8/18 (44%), and the tonsil in 10/25 (40%).

***> Conclusions: This study demonstrates that PrPSc accumulates in lymphoid tissues from pigs challenged intracranially or orally with the CWD agent, and can be detected as early as 4 months after challenge. CWD-infected pigs rarely develop clinical disease and if they do, they do so after a long incubation period. This raises the possibility that CWD-infected pigs could shed prions into their environment long before they develop clinical disease. Furthermore, lymphoid tissues from CWD-infected pigs could present a potential source of CWD infectivity in the animal and human food chains.

https://www.ars.usda.gov/research/publications/publication/?seqNo115=353091

https://www.ars.usda.gov/research/project/?accnNo=432011&fy=2017

https://www.ars.usda.gov/research/publications/publication/?seqNo115=337105

CONFIDENTIAL

EXPERIMENTAL PORCINE SPONGIFORM ENCEPHALOPATHY

LINE TO TAKE

3. If questions on pharmaceuticals are raised at the Press conference, the suggested line to take is as follows:-

"There are no medicinal products licensed for use on the market which make use of UK-derived porcine tissues with which any hypothetical “high risk" ‘might be associated. The results of the recent experimental work at the CSM will be carefully examined by the CSM‘s Working Group on spongiform encephalopathy at its next meeting.

DO Hagger RM 1533 MT Ext 3201

http://web.archive.org/web/20030822054419/www.bseinquiry.gov.uk/files/yb/1990/09/21009001.pdf

While this clearly is a cause for concern we should not jump to the conclusion that this means that pigs will necessarily be infected by bone and meat meal fed by the oral route as is the case with cattle. ...

http://web.archive.org/web/20031026000118/www.bseinquiry.gov.uk/files/yb/1990/08/23004001.pdf

we cannot rule out the possibility that unrecognised subclinical spongiform encephalopathy could be present in British pigs though there is no evidence for this: only with parenteral/implantable pharmaceuticals/devices is the theoretical risk to humans of sufficient concern to consider any action.

http://web.archive.org/web/20030822031154/www.bseinquiry.gov.uk/files/yb/1990/09/10007001.pdf

May I, at the outset, reiterate that we should avoid dissemination of papers relating to this experimental finding to prevent premature release of the information. ...

http://web.archive.org/web/20030822052332/www.bseinquiry.gov.uk/files/yb/1990/09/11005001.pdf

3. It is particularly important that this information is not passed outside the Department, until Ministers have decided how they wish it to be handled. ...

http://web.archive.org/web/20030822052438/www.bseinquiry.gov.uk/files/yb/1990/09/12002001.pdf

But it would be easier for us if pharmaceuticals/devices are not directly mentioned at all. ...

http://web.archive.org/web/20030518170213/www.bseinquiry.gov.uk/files/yb/1990/09/13004001.pdf

Our records show that while some use is made of porcine materials in medicinal products, the only products which would appear to be in a hypothetically ''higher risk'' area are the adrenocorticotrophic hormone for which the source material comes from outside the United Kingdom, namely America China Sweden France and Germany. The products are manufactured by Ferring and Armour. A further product, ''Zenoderm Corium implant'' manufactured by Ethicon, makes use of porcine skin - which is not considered to be a ''high risk'' tissue, but one of its uses is described in the data sheet as ''in dural replacement''. This product is sourced from the United Kingdom.....

http://web.archive.org/web/20030822054419/www.bseinquiry.gov.uk/files/yb/1990/09/21009001.pdf

Sent: Fri, Aug 27, 2021 11:08 am

Subject: Chronic Wasting Disease from pigs is infectious in transgenic mice expressing human PRNP

FRIDAY, AUGUST 27, 2021

Chronic Wasting Disease from pigs is infectious in transgenic mice expressing human PRNP

https://chronic-wasting-disease.blogspot.com/2021/08/chronic-wasting-disease-from-pigs-is.html

https://madporcinedisease.blogspot.com/2021/08/the-agent-of-chronic-wasting-disease.html

FRIDAY, AUGUST 27, 2021

Cattle are highly susceptible to white-tailed deer CWD and mule deer CWD in experimental conditions

https://transmissiblespongiformencephalopathy.blogspot.com/2021/08/cattle-are-highly-susceptible-to-white.html

SUNDAY, DECEMBER 20, 2020

Second passage of chronic wasting disease of mule deer in sheep compared to classical scrapie after intracranial inoculation

https://chronic-wasting-disease.blogspot.com/2020/12/second-passage-of-chronic-wasting.html

THURSDAY, DECEMBER 19, 2019

TSE surveillance statistics exotic species and domestic cats Update December 2019

https://transmissiblespongiformencephalopathy.blogspot.com/2019/12/tse-surveillance-statistics-exotic.html

***> CHRONIC WASTING DISEASE TSE PRP HUMANS ZOONOSIS ZOONOTIC <***

WHAT WE HAVE HERE, IS A LACK OF COMMUNICATION!

seems to me we might have another zoonotic tse prion disease, OR multiple new tse prion zoonotic diseases, that no one wants to talk about, and that's bad...terry

i thought i might share some news about cwd zoonosis that i got, that i cannot share or post to the public yet, i promised for various reasons, one that it will cause a shit storm for sure, but it was something i really already knew from previous studies, but, i was told that ;

==================

''As you can imagine, 2 and 5 (especially 5) may raise alarms. The evidence we have for 4 are not as strong or tight as I would like to have. At this point, please do not post any of the points publicly yet, but you can refer to points 1-3 in private discussions and all 5 points when discussing with relevant public officials to highlight the long-term risks of CWD zoonosis.''

====================

so, i figure your as about as official as it gets, and i think this science is extremely important for you to know and to converse about with your officials. it's about to burn a whole in my pocket. this is about as close as it will ever get for cwd zoonosis to be proven in my time, this and what Canada Czub et al found with the Macaques, plus an old study from cjd surveillance unit back that showed cjd and a 9% increase in risk from folks that eat venison, i will post all this below for your files Sir. i remember back in the BSE nvCJD days, from when the first BSE case in bovine was confirmed around 1984 maybe 83, i forget the good vets named that screwed it up first, Carol something, but from 83ish to 95 96 when nvCJD was linked to humans from BSE in cattle, so that took 10 to 15 years. hell, at that rate, especially with Texas and cwd zoonsis, hell, i'll be dead before it's official, if ever, so here ya go Sir. there was a grant study on cwd zoonosis that had been going on for some time, i followed it over the years, then the grant date for said study had expired, so, i thought i would write the good Professor about said study i.e. Professor Kong, CWRU et al. i will post the grant study abstract first, and then after that, what reply i got back, about said study that i was told not to post/publish...

CWD ZOONOSIS GRANT FIRST;

===============

Cervid to human prion transmission

Kong, Qingzhong

Case Western Reserve University, Cleveland, OH, United States

Abstract Prion disease is transmissible and invariably fatal. Chronic wasting disease (CWD) is the prion disease affecting deer, elk and moose, and it is a widespread and expanding epidemic affecting 22 US States and 2 Canadian provinces so far. CWD poses the most serious zoonotic prion transmission risks in North America because of huge venison consumption (>6 million deer/elk hunted and consumed annually in the USA alone), significant prion infectivity in muscles and other tissues/fluids from CWD-affected cervids, and usually high levels of individual exposure to CWD resulting from consumption of the affected animal among often just family and friends. However, we still do not know whether CWD prions can infect humans in the brain or peripheral tissues or whether clinical/asymptomatic CWD zoonosis has already occurred, and we have no essays to reliably detect CWD infection in humans. We hypothesize that: (1) The classic CWD prion strain can infect humans at low levels in the brain and peripheral lymphoid tissues; (2) The cervid-to-human transmission barrier is dependent on the cervid prion strain and influenced by the host (human) prion protein (PrP) primary sequence; (3) Reliable essays can be established to detect CWD infection in humans; and (4) CWD transmission to humans has already occurred. We will test these hypotheses in 4 Aims using transgenic (Tg) mouse models and complementary in vitro approaches.

Aim 1 will prove that the classical CWD strain may infect humans in brain or peripheral lymphoid tissues at low levels by conducting systemic bioassays in a set of humanized Tg mouse lines expressing common human PrP variants using a number of CWD isolates at varying doses and routes. Experimental human CWD samples will also be generated for Aim 3.

Aim 2 will test the hypothesis that the cervid-to-human prion transmission barrier is dependent on prion strain and influenced by the host (human) PrP sequence by examining and comparing the transmission efficiency and phenotypes of several atypical/unusual CWD isolates/strains as well as a few prion strains from other species that have adapted to cervid PrP sequence, utilizing the same panel of humanized Tg mouse lines as in Aim 1.

Aim 3 will establish reliable essays for detection and surveillance of CWD infection in humans by examining in details the clinical, pathological, biochemical and in vitro seeding properties of existing and future experimental human CWD samples generated from Aims 1-2 and compare them with those of common sporadic human Creutzfeldt-Jakob disease (sCJD) prions.

Aim 4 will attempt to detect clinical CWD-affected human cases by examining a significant number of brain samples from prion-affected human subjects in the USA and Canada who have consumed venison from CWD-endemic areas utilizing the criteria and essays established in Aim 3. The findings from this proposal will greatly advance our understandings on the potential and characteristics of cervid prion transmission in humans, establish reliable essays for CWD zoonosis and potentially discover the first case(s) of CWD infection in humans.

Public Health Relevance There are significant and increasing human exposure to cervid prions because chronic wasting disease (CWD, a widespread and highly infectious prion disease among deer and elk in North America) continues spreading and consumption of venison remains popular, but our understanding on cervid-to-human prion transmission is still very limited, raising public health concerns. This proposal aims to define the zoonotic risks of cervid prions and set up and apply essays to detect CWD zoonosis using mouse models and in vitro methods. The findings will greatly expand our knowledge on the potentials and characteristics of cervid prion transmission in humans, establish reliable essays for such infections and may discover the first case(s) of CWD infection in humans.

Funding Agency Agency National Institute of Health (NIH) Institute National Institute of Neurological Disorders and Stroke (NINDS) Type Research Project (R01) Project # 1R01NS088604-01A1 Application # 9037884 Study Section Cellular and Molecular Biology of Neurodegeneration Study Section (CMND) Program Officer Wong, May Project Start 2015-09-30 Project End 2019-07-31 Budget Start 2015-09-30 Budget End 2016-07-31 Support Year 1 Fiscal Year 2015 Total Cost $337,507 Indirect Cost $118,756

snip...

https://grantome.com/grant/NIH/R01-NS088604-01A1#panel-abstract

Professor Kongs reply to me just this month about above grant study that has NOT been published in peer reveiw yet...

=================================

Here is a brief summary of our findings:

snip...can't post, made a promise...tss

On Sat, Apr 3, 2021 at 12:19 PM Terry Singeltary <flounder9@verizon.net> wrote:

snip...

end...tss

==============

CWD ZOONOSIS THE FULL MONTY TO DATE

International Conference on Emerging Diseases, Outbreaks & Case Studies & 16th Annual Meeting on Influenza March 28-29, 2018 | Orlando, USA

Qingzhong Kong

Case Western Reserve University School of Medicine, USA

Zoonotic potential of chronic wasting disease prions from cervids

Chronic wasting disease (CWD) is the prion disease in cervids (mule deer, white-tailed deer, American elk, moose, and reindeer). It has become an epidemic in North America, and it has been detected in the Europe (Norway) since 2016. The widespread CWD and popular hunting and consumption of cervid meat and other products raise serious public health concerns, but questions remain on human susceptibility to CWD prions, especially on the potential difference in zoonotic potential among the various CWD prion strains. We have been working to address this critical question for well over a decade. We used CWD samples from various cervid species to inoculate transgenic mice expressing human or elk prion protein (PrP). We found infectious prions in the spleen or brain in a small fraction of CWD-inoculated transgenic mice expressing human PrP, indicating that humans are not completely resistant to CWD prions; this finding has significant ramifications on the public health impact of CWD prions. The influence of cervid PrP polymorphisms, the prion strain dependence of CWD-to-human transmission barrier, and the characterization of experimental human CWD prions will be discussed.

Speaker Biography Qingzhong Kong has completed his PhD from the University of Massachusetts at Amherst and Post-doctoral studies at Yale University. He is currently an Associate Professor of Pathology, Neurology and Regenerative Medicine. He has published over 50 original research papers in reputable journals (including Science Translational Medicine, JCI, PNAS and Cell Reports) and has been serving as an Editorial Board Member on seven scientific journals. He has multiple research interests, including public health risks of animal prions (CWD of cervids and atypical BSE of cattle), animal modeling of human prion diseases, mechanisms of prion replication and pathogenesis, etiology of sporadic Creutzfeldt-Jacob disease (CJD) in humans, normal cellular PrP in the biology and pathology of multiple brain and peripheral diseases, proteins responsible for the α-cleavage of cellular PrP, as well as gene therapy and DNA vaccination.

qxk2@case.edu

https://www.alliedacademies.org/conference-abstracts-files/zoonotic-potential-of-chronic-wasting-disease-prions-from.pdf

https://prionconference.blogspot.com/2018/02/prion-round-table-conference-2018-may.html

http://prionconference.blogspot.com/2018/02/prion-round-table-conference-2018-may.html

http://prionconference.blogspot.com/

SUNDAY, JULY 25, 2021

North American and Norwegian Chronic Wasting Disease prions exhibit different potential for interspecies transmission and zoonotic risk

''Our data suggest that reindeer and red deer from Norway could be the most transmissible CWD prions to other mammals, whereas North American CWD prions were more prone to generate human prions in vitro.''

https://chronic-wasting-disease.blogspot.com/2021/07/north-american-and-norwegian-chronic.html

MONDAY, JULY 19, 2021

***> U Calgary researchers at work on a vaccine against a fatal infectious disease affecting deer and potentially people

https://chronic-wasting-disease.blogspot.com/2021/07/u-calgary-researchers-at-work-on.html

Prion Conference 2018 Abstracts

BSE aka MAD COW DISEASE, was first discovered in 1984, and it took until 1995 to finally admit that BSE was causing nvCJD, the rest there is history, but that science is still evolving i.e. science now shows that indeed atypical L-type BSE, atypical Nor-98 Scrapie, and typical Scrapie are all zoonosis, zoonotic for humans, there from.

HOW long are we going to wait for Chronic Wasting Disease, CWD TSE Prion of Cervid, and zoonosis, zoonotic tranmission to humans there from?

Studies have shown since 1994 that humans are susceptible to CWD TSE Prion, so, what's the hold up with making CWD a zoonotic zoonosis disease, the iatrogenic transmissions there from is not waiting for someone to make a decision.

Prion Conference 2018 Abstracts

P190 Human prion disease mortality rates by occurrence of chronic wasting disease in freeranging cervids, United States

Abrams JY (1), Maddox RA (1), Schonberger LB (1), Person MK (1), Appleby BS (2), Belay ED (1)

(1) Centers for Disease Control and Prevention (CDC), National Center for Emerging and Zoonotic Infectious Diseases, Atlanta, GA, USA (2) Case Western Reserve University, National Prion Disease Pathology Surveillance Center (NPDPSC), Cleveland, OH, USA.

Background

Chronic wasting disease (CWD) is a prion disease of deer and elk that has been identified in freeranging cervids in 23 US states. While there is currently no epidemiological evidence for zoonotic transmission through the consumption of contaminated venison, studies suggest the CWD agent can cross the species barrier in experimental models designed to closely mimic humans. We compared rates of human prion disease in states with and without CWD to examine the possibility of undetermined zoonotic transmission.

Methods

Death records from the National Center for Health Statistics, case records from the National Prion Disease Pathology Surveillance Center, and additional state case reports were combined to create a database of human prion disease cases from 2003-2015. Identification of CWD in each state was determined through reports of positive CWD tests by state wildlife agencies. Age- and race-adjusted mortality rates for human prion disease, excluding cases with known etiology, were determined for four categories of states based on CWD occurrence: highly endemic (>16 counties with CWD identified in free-ranging cervids); moderately endemic (3-10 counties with CWD); low endemic (1-2 counties with CWD); and no CWD states. States were counted as having no CWD until the year CWD was first identified. Analyses stratified by age, sex, and time period were also conducted to focus on subgroups for which zoonotic transmission would be more likely to be detected: cases <55 years old, male sex, and the latter half of the study (2010-2015).

Results

Highly endemic states had a higher rate of prion disease mortality compared to non-CWD states (rate ratio [RR]: 1.12, 95% confidence interval [CI] = 1.01 - 1.23), as did low endemic states (RR: 1.15, 95% CI = 1.04 - 1.27). Moderately endemic states did not have an elevated mortality rate (RR: 1.05, 95% CI = 0.93 - 1.17). In age-stratified analyses, prion disease mortality rates among the <55 year old population were elevated for moderately endemic states (RR: 1.57, 95% CI = 1.10 – 2.24) while mortality rates were elevated among those ≥55 for highly endemic states (RR: 1.13, 95% CI = 1.02 - 1.26) and low endemic states (RR: 1.16, 95% CI = 1.04 - 1.29). In other stratified analyses, prion disease mortality rates for males were only elevated for low endemic states (RR: 1.27, 95% CI = 1.10 - 1.48), and none of the categories of CWD-endemic states had elevated mortality rates for the latter time period (2010-2015).

Conclusions

While higher prion disease mortality rates in certain categories of states with CWD in free-ranging cervids were noted, additional stratified analyses did not reveal markedly elevated rates for potentially sensitive subgroups that would be suggestive of zoonotic transmission. Unknown confounding factors or other biases may explain state-by-state differences in prion disease mortality.

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P172 Peripheral Neuropathy in Patients with Prion Disease

Wang H(1), Cohen M(1), Appleby BS(1,2)

(1) University Hospitals Cleveland Medical Center, Cleveland, Ohio (2) National Prion Disease Pathology Surveillance Center, Cleveland, Ohio.

Prion disease is a fatal progressive neurodegenerative disease due to deposition of an abnormal protease-resistant isoform of prion protein. Typical symptoms include rapidly progressive dementia, myoclonus, visual disturbance and hallucinations. Interestingly, in patients with prion disease, the abnormal protein canould also be found in the peripheral nervous system. Case reports of prion deposition in peripheral nerves have been reported. Peripheral nerve involvement is thought to be uncommon; however, little is known about the exact prevalence and features of peripheral neuropathy in patients with prion disease.

We reviewed autopsy-proven prion cases from the National Prion Disease Pathology Surveillance Center that were diagnosed between September 2016 to March 2017. We collected information regarding prion protein diagnosis, demographics, comorbidities, clinical symptoms, physical exam, neuropathology, molecular subtype, genetics lab, brain MRI, image and EMG reports. Our study included 104 patients. Thirteen (12.5%) patients had either subjective symptoms or objective signs of peripheral neuropathy. Among these 13 patients, 3 had other known potential etiologies of peripheral neuropathy such as vitamin B12 deficiency or prior chemotherapy. Among 10 patients that had no other clear etiology, 3 (30%) had familial CJD. The most common sCJD subtype was MV1-2 (30%), followed by MM1-2 (20%). The Majority of cases wasere male (60%). Half of them had exposure to wild game. The most common subjective symptoms were tingling and/or numbness of distal extremities. The most common objective finding was diminished vibratory sensation in the feet. Half of them had an EMG with the findings ranging from fasciculations to axonal polyneuropathy or demyelinating polyneuropathy.

Our study provides an overview of the pattern of peripheral neuropathy in patients with prion disease. Among patients with peripheral neuropathy symptoms or signs, majority has polyneuropathy. It is important to document the baseline frequency of peripheral neuropathy in prion diseases as these symptoms may become important when conducting surveillance for potential novel zoonotic prion diseases.

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P177 PrP plaques in methionine homozygous Creutzfeldt-Jakob disease patients as a potential marker of iatrogenic transmission

Abrams JY (1), Schonberger LB (1), Cali I (2), Cohen Y (2), Blevins JE (2), Maddox RA (1), Belay ED (1), Appleby BS (2), Cohen ML (2)

Background

Sporadic Creutzfeldt-Jakob disease (CJD) is widely believed to originate from de novo spontaneous conversion of normal prion protein (PrP) to its pathogenic form, but concern remains that some reported sporadic CJD cases may actually be caused by disease transmission via iatrogenic processes. For cases with methionine homozygosity (CJD-MM) at codon 129 of the PRNP gene, recent research has pointed to plaque-like PrP deposition as a potential marker of iatrogenic transmission for a subset of cases. This phenotype is theorized to originate from specific iatrogenic source CJD types that comprise roughly a quarter of known CJD cases.

Methods

We reviewed scientific literature for studies which described PrP plaques among CJD patients with known epidemiological links to iatrogenic transmission (receipt of cadaveric human grown hormone or dura mater), as well as in cases of reported sporadic CJD. The presence and description of plaques, along with CJD classification type and other contextual factors, were used to summarize the current evidence regarding plaques as a potential marker of iatrogenic transmission. In addition, 523 cases of reported sporadic CJD cases in the US from January 2013 through September 2017 were assessed for presence of PrP plaques.

Results

We identified four studies describing 52 total cases of CJD-MM among either dura mater recipients or growth hormone recipients, of which 30 were identified as having PrP plaques. While sporadic cases were not generally described as having plaques, we did identify case reports which described plaques among sporadic MM2 cases as well as case reports of plaques exclusively in white matter among sporadic MM1 cases. Among the 523 reported sporadic CJD cases, 0 of 366 MM1 cases had plaques, 2 of 48 MM2 cases had kuru plaques, and 4 of 109 MM1+2 cases had either kuru plaques or both kuru and florid plaques. Medical chart review of the six reported sporadic CJD cases with plaques did not reveal clinical histories suggestive of potential iatrogenic transmission.

Conclusions

PrP plaques occur much more frequently for iatrogenic CJD-MM cases compared to sporadic CJDMM cases. Plaques may indicate iatrogenic transmission for CJD-MM cases without a type 2 Western blot fragment. The study results suggest the absence of significant misclassifications of iatrogenic CJD as sporadic. To our knowledge, this study is the first to describe grey matter kuru plaques in apparently sporadic CJD-MM patients with a type 2 Western blot fragment.

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P180 Clinico-pathological analysis of human prion diseases in a brain bank series

Ximelis T (1), Aldecoa I (1,2), Molina-Porcel L (1,3), Grau-Rivera O (4), Ferrer I (5), Nos C (6), Gelpi E (1,7), Sánchez-Valle R (1,4)

(1) Neurological Tissue Bank of the Biobanc-Hospital ClÃnic-IDIBAPS, Barcelona, Spain (2) Pathological Service of Hospital ClÃnic de Barcelona, Barcelona, Spain (3) EAIA Trastorns Cognitius, Centre Emili Mira, Parc de Salut Mar, Barcelona, Spain (4) Department of Neurology of Hospital ClÃnic de Barcelona, Barcelona, Spain (5) Institute of Neuropathology, Hospital Universitari de Bellvitge, Hospitalet de Llobregat, Barcelona (6) General subdirectorate of Surveillance and Response to Emergencies in Public Health, Department of Public Health in Catalonia, Barcelona, Spain (7) Institute of Neurology, Medical University of Vienna, Vienna, Austria.

Background and objective:

The Neurological Tissue Bank (NTB) of the Hospital Clínic-Institut d‘Investigacions Biomèdiques August Pi i Sunyer, Barcelona, Spain is the reference center in Catalonia for the neuropathological study of prion diseases in the region since 2001. The aim of this study is to analyse the characteristics of the confirmed prion diseases registered at the NTB during the last 15 years.

Methods:

We reviewed retrospectively all neuropathologically confirmed cases registered during the period January 2001 to December 2016.

Results:

176 cases (54,3% female, mean age: 67,5 years and age range: 25-86 years) of neuropathological confirmed prion diseases have been studied at the NTB. 152 cases corresponded to sporadic Creutzfeldt-Jakob disease (sCJD), 10 to genetic CJD, 10 to Fatal Familial Insomnia, 2 to GerstmannSträussler-Scheinker disease, and 2 cases to variably protease-sensitive prionopathy (VPSPr). Within sCJD subtypes the MM1 subtype was the most frequent, followed by the VV2 histotype.

Clinical and neuropathological diagnoses agreed in 166 cases (94%). The clinical diagnosis was not accurate in 10 patients with definite prion disease: 1 had a clinical diagnosis of Fronto-temporal dementia (FTD), 1 Niemann-Pick‘s disease, 1 Lewy Body‘s Disease, 2 Alzheimer‘s disease, 1 Cortico-basal syndrome and 2 undetermined dementia. Among patients with VPSPr, 1 had a clinical diagnosis of Amyotrophic lateral sclerosis (ALS) and the other one with FTD.

Concomitant pathologies are frequent in older age groups, mainly AD neuropathological changes were observed in these subjects.

Discussion:

A wide spectrum of human prion diseases have been identified in the NTB being the relative frequencies and main characteristics like other published series. There is a high rate of agreement between clinical and neuropathological diagnoses with prion diseases. These findings show the importance that public health has given to prion diseases during the past 15 years. Continuous surveillance of human prion disease allows identification of new emerging phenotypes. Brain tissue samples from these donors are available to the scientific community. For more information please visit:

http://www.clinicbiobanc.org/banc-teixits-neurologics/mostres/en_index.html

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P192 Prion amplification techniques for the rapid evaluation of surface decontamination procedures

Bruyere-Ostells L (1), Mayran C (1), Belondrade M (1), Boublik Y (2), Haïk S (3), Fournier-Wirth C (1), Nicot S (1), Bougard D (1)

(1) Pathogenesis and control of chronic infections, Etablissement Français du Sang, Inserm, Université de Montpellier, Montpellier, France. (2) Centre de Recherche en Biologie cellulaire de Montpellier, CNRS, Université de Montpellier, Montpellier, France. (3) Inserm U 1127, CNRS UMR 7225, Sorbonne Universités, UPMC Université Paris 06 UMR S 1127, Institut du Cerveau et de la Moelle épinière, ICM, Paris, France.

Aims:

Transmissible Spongiform Encephalopathies (TSE) or prion diseases are a group of incurable and always fatal neurodegenerative disorders including Creutzfeldt-Jakob diseases (CJD) in humans. These pathologies include sporadic (sCJD), genetic and acquired (variant CJD) forms. By the past, sCJD and vCJD were transmitted by different prion contaminated biological materials to patients resulting in more than 400 iatrogenic cases (iCJD). The atypical nature and the biochemical properties of the infectious agent, formed by abnormal prion protein or PrPTSE, make it particularly resistant to conventional decontamination procedures. In addition, PrPTSE is widely distributed throughout the organism before clinical onset in vCJD and can also be detected in some peripheral tissues in sporadic CJD. Risk of iatrogenic transmission of CJD by contaminated medical device remains thus a concern for healthcare facilities. Bioassay is the gold standard method to evaluate the efficacy of prion decontamination procedures but is time-consuming and expensive. Here, we propose to compare in vitro prion amplification techniques: Protein Misfolding Cyclic Amplification (PMCA) and Real-Time Quaking Induced Conversion (RT-QuIC) for the detection of residual prions on surface after decontamination.

Methods:

Stainless steel wires, by mimicking the surface of surgical instruments, were proposed as a carrier model of prions for inactivation studies. To determine the sensitivity of the two amplification techniques on wires (Surf-PMCA and Surf-QuIC), steel wires were therefore contaminated with serial dilutions of brain homogenates (BH) from a 263k infected hamster and from a patient with sCJD (MM1 subtype). We then compared the different standard decontamination procedures including partially and fully efficient treatments by detecting the residual seeding activity on 263K and sCJD contaminated wires. We completed our study by the evaluation of marketed reagents endorsed for prion decontamination.

Results:

The two amplification techniques can detect minute quantities of PrPTSE adsorbed onto a single wire. 8/8 wires contaminated with a 10-6 dilution of 263k BH and 1/6 with the 10-8 dilution are positive with Surf-PMCA. Similar performances were obtained with Surf-QuIC on 263K: 10/16 wires contaminated with 10-6 dilution and 1/8 wires contaminated with 10-8 dilution are positive. Regarding the human sCJD-MM1 prion, Surf-QuIC allows us to detect 16/16 wires contaminated with 10-6 dilutions and 14/16 with 10-7 . Results obtained after decontamination treatments are very similar between 263K and sCJD prions. Efficiency of marketed treatments to remove prions is lower than expected.

Conclusions:

Surf-PMCA and Surf-QuIC are very sensitive methods for the detection of prions on wires and could be applied to prion decontamination studies for rapid evaluation of new treatments. Sodium hypochlorite is the only product to efficiently remove seeding activity of both 263K and sCJD prions.

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WA2 Oral transmission of CWD into Cynomolgus macaques: signs of atypical disease, prion conversion and infectivity in macaques and bio-assayed transgenic mice

Schatzl HM (1, 2), Hannaoui S (1, 2), Cheng Y-C (1, 2), Gilch S (1, 2), Beekes M (3), SchulzSchaeffer W (4), Stahl-Hennig C (5) and Czub S (2, 6)

(1) University of Calgary, Calgary Prion Research Unit, Calgary, Canada (2) University of Calgary, Faculty of Veterinary Medicine, Calgary, Canada, (3) Robert Koch Institute, Berlin, Germany, (4) University of Homburg/Saar, Homburg, Germany, (5) German Primate Center, Goettingen, Germany, (6) Canadian Food Inspection Agency (CFIA), Lethbridge, Canada.

To date, BSE is the only example of interspecies transmission of an animal prion disease into humans. The potential zoonotic transmission of CWD is an alarming issue and was addressed by many groups using a variety of in vitro and in vivo experimental systems. Evidence from these studies indicated a substantial, if not absolute, species barrier, aligning with the absence of epidemiological evidence suggesting transmission into humans. Studies in non-human primates were not conclusive so far, with oral transmission into new-world monkeys and no transmission into old-world monkeys. Our consortium has challenged 18 Cynomolgus macaques with characterized CWD material, focusing on oral transmission with muscle tissue. Some macaques have orally received a total of 5 kg of muscle material over a period of 2 years. After 5-7 years of incubation time some animals showed clinical symptoms indicative of prion disease, and prion neuropathology and PrPSc deposition were found in spinal cord and brain of euthanized animals. PrPSc in immunoblot was weakly detected in some spinal cord materials and various tissues tested positive in RT-QuIC, including lymph node and spleen homogenates. To prove prion infectivity in the macaque tissues, we have intracerebrally inoculated 2 lines of transgenic mice, expressing either elk or human PrP. At least 3 TgElk mice, receiving tissues from 2 different macaques, showed clinical signs of a progressive prion disease and brains were positive in immunoblot and RT-QuIC. Tissues (brain, spinal cord and spleen) from these and preclinical mice are currently tested using various read-outs and by second passage in mice. Transgenic mice expressing human PrP were so far negative for clear clinical prion disease (some mice >300 days p.i.). In parallel, the same macaque materials are inoculated into bank voles. Taken together, there is strong evidence of transmissibility of CWD orally into macaques and from macaque tissues into transgenic mouse models, although with an incomplete attack rate. The clinical and pathological presentation in macaques was mostly atypical, with a strong emphasis on spinal cord pathology. Our ongoing studies will show whether the transmission of CWD into macaques and passage in transgenic mice represents a form of non-adaptive prion amplification, and whether macaque-adapted prions have the potential to infect mice expressing human PrP. The notion that CWD can be transmitted orally into both new-world and old-world non-human primates asks for a careful reevaluation of the zoonotic risk of CWD.

See also poster P103

***> The notion that CWD can be transmitted orally into both new-world and old-world non-human primates asks for a careful reevaluation of the zoonotic risk of CWD.

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WA16 Monitoring Potential CWD Transmission to Humans

Belay ED

Centers for Disease Control and Prevention (CDC), National Center for Emerging and Zoonotic Infectious Diseases, Atlanta, GA, USA.

The spread of chronic wasting disease (CWD) in animals has raised concerns about increasing human exposure to the CWD agent via hunting and venison consumption, potentially facilitating CWD transmission to humans. Several studies have explored this possibility, including limited epidemiologic studies, in vitro experiments, and laboratory studies using various types of animal models. Most human exposures to the CWD agent in the United States would be expected to occur in association with deer and elk hunting in CWD-endemic areas. The Centers for Disease Control and Prevention (CDC) collaborated with state health departments in Colorado, Wisconsin, and Wyoming to identify persons at risk of CWD exposure and to monitor their vital status over time. Databases were established of persons who hunted in Colorado and Wyoming and those who reported consumption of venison from deer that later tested positive in Wisconsin. Information from the databases is periodically cross-checked with mortality data to determine the vital status and causes of death for deceased persons. Long-term follow-up of these hunters is needed to assess their risk of development of a prion disease linked to CWD exposure.

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P166 Characterization of CJD strain profiles in venison consumers and non-consumers from Alberta and Saskatchewan

Stephanie Booth (1,2), Lise Lamoureux (1), Debra Sorensen (1), Jennifer L. Myskiw (1,2), Megan Klassen (1,2), Michael Coulthart (3), Valerie Sim (4)

(1) Zoonotic Diseases and Special Pathogens, National Microbiology Laboratory, Public Health Agency of Canada, Winnipeg (2) Department of Medical Microbiology and Infectious Diseases, University of Manitoba, Winnipeg (3) Canadian CJD Surveillance System, Public Health Agency of Canada, Ottawa (4) Division of Neurology, Department of Medicine Centre for Prions and Protein Folding Diseases, University of Alberta, Edmonton.

Chronic wasting disease (CWD) is spreading rapidly through wild cervid populations in the Canadian provinces of Alberta and Saskatchewan. While this has implications for tourism and hunting, there is also concern over possible zoonotic transmission to humans who eat venison from infected deer. Whilst there is no evidence of any human cases of CWD to date, the Canadian CJD Surveillance System (CJDSS) in Canada is staying vigilant. When variant CJD occurred following exposure to BSE, the unique biochemical fingerprint of the pathologic PrP enabled a causal link to be confirmed. However, we cannot be sure what phenotype human CWD prions would present with, or indeed, whether this would be distinct from that see in sporadic CJD. Therefore we are undertaking a systematic analysis of the molecular diversity of CJD cases of individuals who resided in Alberta and Saskatchewan at their time of death comparing venison consumers and non-consumers, using a variety of clinical, imaging, pathological and biochemical markers. Our initial objective is to develop novel biochemical methodologies that will extend the baseline glycoform and genetic polymorphism typing that is already completed by the CJDSS. Firstly, we are reviewing MRI, EEG and pathology information from over 40 cases of CJD to select clinically affected areas for further investigation. Biochemical analysis will include assessment of the levels of protease sensitive and resistant prion protein, glycoform typing using 2D gel electrophoresis, testing seeding capabilities and kinetics of aggregation by quaking-induced conversion, and determining prion oligomer size distributions with asymmetric flow field fractionation with in-line light scattering. Progress and preliminary data will be presented. Ultimately, we intend to further define the relationship between PrP structure and disease phenotype and establish a baseline for the identification of future atypical CJD cases that may arise as a result of exposure to CWD.

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Source Prion Conference 2018 Abstracts

http://transmissiblespongiformencephalopathy.blogspot.com/2018/05/prion-2018-may-22-25-2018-santiago-de.html

http://chronic-wasting-disease.blogspot.com/2018/07/oral-transmission-of-cwd-into.html

http://prionconference.blogspot.com/2018/

Volume 24, Number 8—August 2018 Research Susceptibility of Human Prion Protein to Conversion by Chronic Wasting Disease Prions

Marcelo A. BarriaComments to Author , Adriana Libori, Gordon Mitchell, and Mark W. Head Author affiliations: National CJD Research and Surveillance Unit, University of Edinburgh, Edinburgh, Scotland, UK (M.A. Barria, A. Libori, M.W. Head); National and OIE Reference Laboratory for Scrapie and CWD, Canadian Food Inspection Agency, Ottawa, Ontario, Canada (G. Mitchell)

Abstract Chronic wasting disease (CWD) is a contagious and fatal neurodegenerative disease and a serious animal health issue for deer and elk in North America. The identification of the first cases of CWD among free-ranging reindeer and moose in Europe brings back into focus the unresolved issue of whether CWD can be zoonotic like bovine spongiform encephalopathy. We used a cell-free seeded protein misfolding assay to determine whether CWD prions from elk, white-tailed deer, and reindeer in North America can convert the human prion protein to the disease-associated form. We found that prions can convert, but the efficiency of conversion is affected by polymorphic variation in the cervid and human prion protein genes. In view of the similarity of reindeer, elk, and white-tailed deer in North America to reindeer, red deer, and roe deer, respectively, in Europe, a more comprehensive and thorough assessment of the zoonotic potential of CWD might be warranted.

snip...

Discussion Characterization of the transmission properties of CWD and evaluation of their zoonotic potential are important for public health purposes. Given that CWD affects several members of the family Cervidae, it seems reasonable to consider whether the zoonotic potential of CWD prions could be affected by factors such as CWD strain, cervid species, geographic location, and Prnp–PRNP polymorphic variation. We have previously used an in vitro conversion assay (PMCA) to investigate the susceptibility of the human PrP to conversion to its disease-associated form by several animal prion diseases, including CWD (15,16,22). The sensitivity of our molecular model for the detection of zoonotic conversion depends on the combination of 1) the action of proteinase K to degrade the abundant human PrPC that constitutes the substrate while only N terminally truncating any human PrPres produced and 2) the presence of the 3F4 epitope on human but not cervid PrP. In effect, this degree of sensitivity means that any human PrPres formed during the PMCA reaction can be detected down to the limit of Western blot sensitivity. In contrast, if other antibodies that detect both cervid and human PrP are used, such as 6H4, then newly formed human PrPres must be detected as a measurable increase in PrPres over the amount remaining in the reaction product from the cervid seed. Although best known for the efficient amplification of prions in research and diagnostic contexts, the variation of the PMCA method employed in our study is optimized for the definitive detection of zoonotic reaction products of inherently inefficient conversion reactions conducted across species barriers. By using this system, we previously made and reported the novel observation that elk CWD prions could convert human PrPC from human brain and could also convert recombinant human PrPC expressed in transgenic mice and eukaryotic cell cultures (15).

A previous publication suggested that mule deer PrPSc was unable to convert humanized transgenic substrate in PMCA assays (23) and required a further step of in vitro conditioning in deer substrate PMCA before it was able to cross the deer–human molecular barrier (24). However, prions from other species, such as elk (15) and reindeer affected by CWD, appear to be compatible with the human protein in a single round of amplification (as shown in our study). These observations suggest that different deer species affected by CWD could present differing degrees of the olecular compatibility with the normal form of human PrP.

The contribution of the polymorphism at codon 129 of the human PrP gene has been extensively studied and is recognized as a risk factor for Creutzfeldt-Jakob disease (4). In cervids, the equivalent codon corresponds to the position 132 encoding methionine or leucine. This polymorphism in the elk gene has been shown to play an important role in CWD susceptibility (25,26). We have investigated the effect of this cervid Prnp polymorphism on the conversion of the humanized transgenic substrate according to the variation in the equivalent PRNP codon 129 polymorphism. Interestingly, only the homologs methionine homozygous seed–substrate reactions could readily convert the human PrP, whereas the heterozygous elk PrPSc was unable to do so, even though comparable amounts of PrPres were used to seed the reaction. In addition, we observed only low levels of human PrPres formation in the reactions seeded with the homozygous methionine (132 MM) and the heterozygous (132 ML) seeds incubated with the other 2 human polymorphic substrates (129 MV and 129 VV). The presence of the amino acid leucine at position 132 of the elk Prnp gene has been attributed to a lower degree of prion conversion compared with methionine on the basis of experiments in mice made transgenic for these polymorphic variants (26). Considering the differences observed for the amplification of the homozygous human methionine substrate by the 2 polymorphic elk seeds (MM and ML), reappraisal of the susceptibility of human PrPC by the full range of cervid polymorphic variants affected by CWD would be warranted.

In light of the recent identification of the first cases of CWD in Europe in a free-ranging reindeer (R. tarandus) in Norway (2), we also decided to evaluate the in vitro conversion potential of CWD in 2 experimentally infected reindeer (18). Formation of human PrPres was readily detectable after a single round of PMCA, and in all 3 humanized polymorphic substrates (MM, MV, and VV). This finding suggests that CWD prions from reindeer could be more compatible with human PrPC generally and might therefore present a greater risk for zoonosis than, for example, CWD prions from white-tailed deer. A more comprehensive comparison of CWD in the affected species, coupled with the polymorphic variations in the human and deer PRNP–Prnp genes, in vivo and in vitro, will be required before firm conclusions can be drawn. Analysis of the Prnp sequence of the CWD reindeer in Norway was reported to be identical to the specimens used in our study (2). This finding raises the possibility of a direct comparison of zoonotic potential between CWD acquired in the wild and that produced in a controlled laboratory setting. (Table).

The prion hypothesis proposes that direct molecular interaction between PrPSc and PrPC is necessary for conversion and prion replication. Accordingly, polymorphic variants of the PrP of host and agent might play a role in determining compatibility and potential zoonotic risk. In this study, we have examined the capacity of the human PrPC to support in vitro conversion by elk, white-tailed deer, and reindeer CWD PrPSc. Our data confirm that elk CWD prions can convert the human PrPC, at least in vitro, and show that the homologous PRNP polymorphisms at codon 129 and 132 in humans and cervids affect conversion efficiency. Other species affected by CWD, particularly caribou or reindeer, also seem able to convert the human PrP. It will be important to determine whether other polymorphic variants found in other CWD-affected Cervidae or perhaps other factors (17) exert similar effects on the ability to convert human PrP and thus affect their zoonotic potential.

Dr. Barria is a research scientist working at the National CJD Research and Surveillance Unit, University of Edinburgh. His research has focused on understanding the molecular basis of a group of fatal neurologic disorders called prion diseases.

Acknowledgments We thank Aru Balachandran for originally providing cervid brain tissues, Abigail Diack and Jean Manson for providing mouse brain tissue, and James Ironside for his critical reading of the manuscript at an early stage.

This report is independent research commissioned and funded by the United Kingdom’s Department of Health Policy Research Programme and the Government of Scotland. The views expressed in this publication are those of the authors and not necessarily those of the Department of Health or the Government of Scotland.

Author contributions: The study was conceived and designed by M.A.B. and M.W.H. The experiments were conducted by M.A.B. and A.L. Chronic wasting disease brain specimens were provided by G.M. The manuscript was written by M.A.B. and M.W.H. All authors contributed to the editing and revision of the manuscript.

https://wwwnc.cdc.gov/eid/article/24/8/16-1888_article

https://www.ed.ac.uk/clinical-brain-sciences/news/news-jul-dec-2018/cwd-prions-human-conversion

Prion 2017 Conference Abstracts

First evidence of intracranial and peroral transmission of Chronic Wasting Disease (CWD) into Cynomolgus macaques: a work in progress Stefanie Czub1, Walter Schulz-Schaeffer2, Christiane Stahl-Hennig3, Michael Beekes4, Hermann Schaetzl5 and Dirk Motzkus6 1

University of Calgary Faculty of Veterinary Medicine/Canadian Food Inspection Agency; 2Universitatsklinikum des Saarlandes und Medizinische Fakultat der Universitat des Saarlandes; 3 Deutsches Primaten Zentrum/Goettingen; 4 Robert-Koch-Institut Berlin; 5 University of Calgary Faculty of Veterinary Medicine; 6 presently: Boehringer Ingelheim Veterinary Research Center; previously: Deutsches Primaten Zentrum/Goettingen

This is a progress report of a project which started in 2009.

21 cynomolgus macaques were challenged with characterized CWD material from white-tailed deer (WTD) or elk by intracerebral (ic), oral, and skin exposure routes. Additional blood transfusion experiments are supposed to assess the CWD contamination risk of human blood product. Challenge materials originated from symptomatic cervids for ic, skin scarification and partially per oral routes (WTD brain). Challenge material for feeding of muscle derived from preclinical WTD and from preclinical macaques for blood transfusion experiments. We have confirmed that the CWD challenge material contained at least two different CWD agents (brain material) as well as CWD prions in muscle-associated nerves.

Here we present first data on a group of animals either challenged ic with steel wires or per orally and sacrificed with incubation times ranging from 4.5 to 6.9 years at postmortem. Three animals displayed signs of mild clinical disease, including anxiety, apathy, ataxia and/or tremor. In four animals wasting was observed, two of those had confirmed diabetes. All animals have variable signs of prion neuropathology in spinal cords and brains and by supersensitive IHC, reaction was detected in spinal cord segments of all animals. Protein misfolding cyclic amplification (PMCA), real-time quaking-induced conversion (RT-QuiC) and PET-blot assays to further substantiate these findings are on the way, as well as bioassays in bank voles and transgenic mice.

At present, a total of 10 animals are sacrificed and read-outs are ongoing. Preclinical incubation of the remaining macaques covers a range from 6.4 to 7.10 years. Based on the species barrier and an incubation time of > 5 years for BSE in macaques and about 10 years for scrapie in macaques, we expected an onset of clinical disease beyond 6 years post inoculation.

PRION 2017 DECIPHERING NEURODEGENERATIVE DISORDERS ABSTRACTS REFERENCE

8. Even though human TSE‐exposure risk through consumption of game from European cervids can be assumed to be minor, if at all existing, no final conclusion can be drawn due to the overall lack of scientific data. In particular the US data do not clearly exclude the possibility of human (sporadic or familial) TSE development due to consumption of venison. The Working Group thus recognizes a potential risk to consumers if a TSE would be present in European cervids. It might be prudent considering appropriate measures to reduce such a risk, e.g. excluding tissues such as CNS and lymphoid tissues from the human food chain, which would greatly reduce any potential risk for consumers. However, it is stressed that currently, no data regarding a risk of TSE infections from cervid products are available.

https://efsa.onlinelibrary.wiley.com/doi/full/10.2903/j.efsa.2018.5132

SATURDAY, FEBRUARY 23, 2019

Chronic Wasting Disease CWD TSE Prion and THE FEAST 2003 CDC an updated review of the science 2019

https://chronic-wasting-disease.blogspot.com/2019/02/chronic-wasting-disease-cwd-tse-prion.html

TUESDAY, NOVEMBER 04, 2014

Six-year follow-up of a point-source exposure to CWD contaminated venison in an Upstate New York community: risk behaviours and health outcomes 2005–2011

Authors, though, acknowledged the study was limited in geography and sample size and so it couldn't draw a conclusion about the risk to humans. They recommended more study. Dr. Ermias Belay was the report's principal author but he said New York and Oneida County officials are following the proper course by not launching a study. "There's really nothing to monitor presently. No one's sick," Belay said, noting the disease's incubation period in deer and elk is measured in years. "

http://chronic-wasting-disease.blogspot.com/2014/11/six-year-follow-up-of-point-source.html

Transmission Studies

Mule deer transmissions of CWD were by intracerebral inoculation and compared with natural cases {the following was written but with a single line marked through it ''first passage (by this route)}....TSS

resulted in a more rapidly progressive clinical disease with repeated episodes of synocopy ending in coma. One control animal became affected, it is believed through contamination of inoculum (?saline). Further CWD transmissions were carried out by Dick Marsh into ferret, mink and squirrel monkey. Transmission occurred in ALL of these species with the shortest incubation period in the ferret.

snip....

https://web.archive.org/web/20090506002237/http://www..bseinquiry.gov.uk/files/mb/m11b/tab01.pdf

Prion Infectivity in Fat of Deer with Chronic Wasting Disease▿

Brent Race#, Kimberly Meade-White#, Richard Race and Bruce Chesebro* + Author Affiliations

In mice, prion infectivity was recently detected in fat. Since ruminant fat is consumed by humans and fed to animals, we determined infectivity titers in fat from two CWD-infected deer. Deer fat devoid of muscle contained low levels of CWD infectivity and might be a risk factor for prion infection of other species.

http://jvi.asm.org/content/83/18/9608.full

Prions in Skeletal Muscles of Deer with Chronic Wasting Disease

Here bioassays in transgenic mice expressing cervid prion protein revealed the presence of infectious prions in skeletal muscles of CWD-infected deer, demonstrating that humans consuming or handling meat from CWD-infected deer are at risk to prion exposure.

http://science.sciencemag.org/content/311/5764/1117..long

*** now, let’s see what the authors said about this casual link, personal communications years ago, and then the latest on the zoonotic potential from CWD to humans from the TOKYO PRION 2016 CONFERENCE.

see where it is stated NO STRONG evidence. so, does this mean there IS casual evidence ???? “Our conclusion stating that we found no strong evidence of CWD transmission to humans”

From: TSS

Subject: CWD aka MAD DEER/ELK TO HUMANS ???

Date: September 30, 2002 at 7:06 am PST

From: "Belay, Ermias"

To: Cc: "Race, Richard (NIH)" ; ; "Belay, Ermias"

Sent: Monday, September 30, 2002 9:22 AM

Subject: RE: TO CDC AND NIH - PUB MED- 3 MORE DEATHS - CWD - YOUNG HUNTERS

Dear Sir/Madam,

In the Archives of Neurology you quoted (the abstract of which was attached to your email), we did not say CWD in humans will present like variant CJD.. That assumption would be wrong. I encourage you to read the whole article and call me if you have questions or need more clarification (phone: 404-639-3091). Also, we do not claim that "no-one has ever been infected with prion disease from eating venison." Our conclusion stating that we found no strong evidence of CWD transmission to humans in the article you quoted or in any other forum is limited to the patients we investigated.

Ermias Belay, M.D. Centers for Disease Control and Prevention

-----Original Message-----

From: Sent: Sunday, September 29, 2002 10:15 AM

To: rr26k@nih.gov; rrace@niaid.nih.gov; ebb8@CDC.GOV

Subject: TO CDC AND NIH - PUB MED- 3 MORE DEATHS - CWD - YOUNG HUNTERS

Sunday, November 10, 2002 6:26 PM .......snip........end..............TSS

Thursday, April 03, 2008

A prion disease of cervids: Chronic wasting disease 2008 1: Vet Res. 2008 Apr 3;39(4):41 A prion disease of cervids: Chronic wasting disease Sigurdson CJ.

snip...

*** twenty-seven CJD patients who regularly consumed venison were reported to the Surveillance Center***,

snip... full text ;

http://chronic-wasting-disease.blogspot.com/2008/04/prion-disease-of-cervids-chronic.html

> However, to date, no CWD infections have been reported in people.

sporadic, spontaneous CJD, 85%+ of all human TSE, did not just happen. never in scientific literature has this been proven.

if one looks up the word sporadic or spontaneous at pubmed, you will get a laundry list of disease that are classified in such a way;

sporadic = 54,983 hits https://www.ncbi.nlm.nih.gov/pubmed/?term=sporadic

spontaneous = 325,650 hits https://www.ncbi.nlm.nih.gov/pubmed/?term=spontaneous

key word here is 'reported'. science has shown that CWD in humans will look like sporadic CJD. SO, how can one assume that CWD has not already transmitted to humans? they can't, and it's as simple as that. from all recorded science to date, CWD has already transmitted to humans, and it's being misdiagnosed as sporadic CJD. ...terry

*** LOOKING FOR CWD IN HUMANS AS nvCJD or as an ATYPICAL CJD, LOOKING IN ALL THE WRONG PLACES $$$ ***

> However, to date, no CWD infections have been reported in people.

key word here is ‘reported’. science has shown that CWD in humans will look like sporadic CJD. SO, how can one assume that CWD has not already transmitted to humans? they can’t, and it’s as simple as that. from all recorded science to date, CWD has already transmitted to humans, and it’s being misdiagnosed as sporadic CJD. …terry

*** LOOKING FOR CWD IN HUMANS AS nvCJD or as an ATYPICAL CJD, LOOKING IN ALL THE WRONG PLACES $$$ ***

*** These results would seem to suggest that CWD does indeed have zoonotic potential, at least as judged by the compatibility of CWD prions and their human PrPC target. Furthermore, extrapolation from this simple in vitro assay suggests that if zoonotic CWD occurred, it would most likely effect those of the PRNP codon 129-MM genotype and that the PrPres type would be similar to that found in the most common subtype of sCJD (MM1).***

http://www.tandfonline.com/doi/full/10.4161/pri.28124?src=recsys

http://www.tandfonline.com/doi/pdf/10.4161/pri.28124?needAccess=true

https://wwwnc.cdc.gov/eid/article/20/1/13-0858_article

CWD TSE PRION AND ZOONOTIC, ZOONOSIS, POTENTIAL

Subject: Re: DEER SPONGIFORM ENCEPHALOPATHY SURVEY & HOUND STUDY

Date: Fri, 18 Oct 2002 23:12:22 +0100

From: Steve Dealler

Reply-To: Bovine Spongiform Encephalopathy Organization: Netscape Online member

To: BSE-L@ References: <3daf5023 .4080804="" wt.net="">

Dear Terry,

An excellent piece of review as this literature is desparately difficult to get back from Government sites.

What happened with the deer was that an association between deer meat eating and sporadic CJD was found in about 1993. The evidence was not great but did not disappear after several years of asking CJD cases what they had eaten. I think that the work into deer disease largely stopped because it was not helpful to the UK industry...and no specific cases were reported. Well, if you dont look adequately like they are in USA currenly then you wont find any!

Steve Dealler ===============

https://caninespongiformencephalopathy.blogspot.com/2010/03/canine-spongiform-encephalopathy-aka.html

''The association between venison eating and risk of CJD shows similar pattern, with regular venison eating associated with a 9 FOLD INCREASE IN RISK OF CJD (p = 0.04).''

CREUTZFELDT JAKOB DISEASE SURVEILLANCE IN THE UNITED KINGDOM THIRD ANNUAL REPORT AUGUST 1994

Consumption of venison and veal was much less widespread among both cases and controls. For both of these meats there was evidence of a trend with increasing frequency of consumption being associated with increasing risk of CJD. (not nvCJD, but sporadic CJD...tss) These associations were largely unchanged when attention was restricted to pairs with data obtained from relatives. ...

Table 9 presents the results of an analysis of these data.

There is STRONG evidence of an association between ‘’regular’’ veal eating and risk of CJD (p = .0.01).

Individuals reported to eat veal on average at least once a year appear to be at 13 TIMES THE RISK of individuals who have never eaten veal.

There is, however, a very wide confidence interval around this estimate. There is no strong evidence that eating veal less than once per year is associated with increased risk of CJD (p = 0.51).

The association between venison eating and risk of CJD shows similar pattern, with regular venison eating associated with a 9 FOLD INCREASE IN RISK OF CJD (p = 0.04).

There is some evidence that risk of CJD INCREASES WITH INCREASING FREQUENCY OF LAMB EATING (p = 0.02).

The evidence for such an association between beef eating and CJD is weaker (p = 0.14). When only controls for whom a relative was interviewed are included, this evidence becomes a little STRONGER (p = 0.08).

snip...

It was found that when veal was included in the model with another exposure, the association between veal and CJD remained statistically significant (p = < 0.05 for all exposures), while the other exposures ceased to be statistically significant (p = > 0.05).

snip...

In conclusion, an analysis of dietary histories revealed statistical associations between various meats/animal products and INCREASED RISK OF CJD. When some account was taken of possible confounding, the association between VEAL EATING AND RISK OF CJD EMERGED AS THE STRONGEST OF THESE ASSOCIATIONS STATISTICALLY. ...

snip...

In the study in the USA, a range of foodstuffs were associated with an increased risk of CJD, including liver consumption which was associated with an apparent SIX-FOLD INCREASE IN THE RISK OF CJD. By comparing the data from 3 studies in relation to this particular dietary factor, the risk of liver consumption became non-significant with an odds ratio of 1.2 (PERSONAL COMMUNICATION, PROFESSOR A. HOFMAN. ERASMUS UNIVERSITY, ROTTERDAM). (???...TSS)

snip...see full report ;

http://web.archive.org/web/20090506050043/http://www.bseinquiry.gov.uk/files/yb/1994/08/00004001.pdf

http://web.archive.org/web/20090506050007/http://www.bseinquiry.gov.uk/files/yb/1994/10/00003001.pdf

http://web.archive.org/web/20090506050244/http://www.bseinquiry.gov.uk/files/yb/1994/07/00001001.pdf

Stephen Dealler is a consultant medical microbiologist deal@airtime.co.uk

BSE Inquiry Steve Dealler

Management In Confidence

BSE: Private Submission of Bovine Brain Dealler

snip...see full text;

MONDAY, FEBRUARY 25, 2019

***> MAD DOGS AND ENGLISHMEN BSE, SCRAPIE, CWD, CJD, TSE PRION A REVIEW 2019

https://bseinquiry.blogspot.com/2019/02/mad-dogs-and-englishmen-bse-scrapie-cwd.html

***> ''The association between venison eating and risk of CJD shows similar pattern, with regular venison eating associated with a 9 FOLD INCREASE IN RISK OF CJD (p = 0.04).''

***> In conclusion, sensory symptoms and loss of reflexes in Gerstmann-Sträussler-Scheinker syndrome can be explained by neuropathological changes in the spinal cord. We conclude that the sensory symptoms and loss of lower limb reflexes in Gerstmann-Sträussler-Scheinker syndrome is due to pathology in the caudal spinal cord. <***

***> The clinical and pathological presentation in macaques was mostly atypical, with a strong emphasis on spinal cord pathology.<***

***> The notion that CWD can be transmitted orally into both new-world and old-world non-human primates asks for a careful reevaluation of the zoonotic risk of CWD. <***

***> All animals have variable signs of prion neuropathology in spinal cords and brains and by supersensitive IHC, reaction was detected in spinal cord segments of all animals.<***

***> In particular the US data do not clearly exclude the possibility of human (sporadic or familial) TSE development due to consumption of venison. The Working Group thus recognizes a potential risk to consumers if a TSE would be present in European cervids.'' Scientific opinion on chronic wasting disease (II) <***

https://familialcjdtseprion.blogspot.com/2019/02/cwd-gss-tse-prion-spinal-cord-confucius.html

North American and Norwegian Chronic Wasting Disease prions exhibit different potential for interspecies transmission and zoonotic risk

Sandra Pritzkow1,*, Damian Gorski1,*, Frank Ramirez1 , Glenn C. Telling2 , Sylvie L. Benestad3 and Claudio Soto1,#

1 Mitchell Center for Alzheimer's disease and related Brain disorders, Department of Neurology, University of Texas McGovern Medical School at Houston, Texas, USA 2 Prion Research Center, Department of Microbiology, Immunology and Pathology, Colorado State University, Fort Collins, Colorado, USA 3 Norwegian Veterinary Institute, OIE Reference Laboratory for CWD, Oslo, Norway.

Summary: We investigated the in vitro spillover and zoonotic potential of CWD from various cervid species. Our results suggest that Norway CWD prions have a higher potential to infect other animals, but NorthAmerican CWD appear more prone to generate human prions.

The current evidence for CWD transmission to humans is controversial; indeed, while transgenic mice expressing human PrP did not develop disease when challenged with CWD prions in various laboratories [6-8, 41], experimental inoculation of CWD into squirrel monkeys produced disease [9, 10]. Studies in macaques, which are phylogenetically closer to humans than squirrel monkeys [45] have shown mixed results. A study from Czub and colleagues found that CWD prions can induce disease and pathologic abnormalities typical of prion disease in macaques exposed to CWD prions, even by oral inoculation of muscle tissue from cervids affected by CWD [46]. However, a different study found no evidence for prion disease in macaques inoculated with CWD [47]. To assess the cervid/human species barrier, we previously used PMCA to determine prion replication in vitro. We found that, after stabilization by successive passages in deer PrPC, PrPSc from CWD infected deer can convert human PrPC into a novel form of PrPSc [13]. Our current study to evaluate in vitro zoonotic potential of various CWD prions showed that although the cervid/human barrier is large, we were able to observe generation of human PrPSc with some specific CWD strains in a second round of PMCA (Fig. 5). The three North American CWD isolates were capable to sustain generation of human PrPSc, with white-tailed deer showing the highest efficiency. Conversely, none of the three Norway CWD isolates generated any detectable PrPSc signal up to the second round of PMCA. This data suggest that North American CWD prions might be of a greater risk to humans than the infected animals in Northern Europe. We speculate that these differences might be due to Norwegian CWD being less stable prion strains as compared to North American CWD, which have had longer time to replicate in cervids and become stabilized through many rounds of natural infection. Our findings may provide important information to understand the diversity of natural CWD prion strains in different animals across distinct geographical areas and their consequences for the spillover into other animal species, including humans.

https://academic.oup.com/jid/advance-article-abstract/doi/10.1093/infdis/jiab385/6327572?redirectedFrom=fulltext

MONDAY, JULY 19, 2021

U Calgary researchers at work on a vaccine against a fatal infectious disease affecting deer and potentially people

https://chronic-wasting-disease.blogspot.com/2021/07/u-calgary-researchers-at-work-on.html

TUESDAY, JULY 13, 2021

Chronic Wasting Disease and the Canadian Agriculture and Agri-food Sectors Current Knowledge Risks and Policy Options

''The science is progressing on the possibility of transmission of CWD to humans through oral transmission, but the complete assessment of this possibility remains to be done.''

https://chronic-wasting-disease.blogspot.com/2021/07/chronic-wasting-disease-and-canadian.html

MONDAY, DECEMBER 16, 2019

Chronic Wasting Disease CWD TSE Prion aka mad cow type disease in cervid Zoonosis Update

***> ''In particular the US data do not clearly exclude the possibility of human (sporadic or familial) TSE development due to consumption of venison. The Working Group thus recognizes a potential risk to consumers if a TSE would be present in European cervids.'' Scientific opinion on chronic wasting disease (II) <***

What if?

https://chronic-wasting-disease.blogspot.com/2019/12/chronic-wasting-disease-cwd-tse-prion.html

TUESDAY, MAY 11, 2021

A Unique Presentation of Creutzfeldt-Jakob Disease in a Patient Consuming Deer Antler Velvet

https://creutzfeldt-jakob-disease.blogspot.com/2021/05/a-unique-presentation-of-creutzfeldt.html

Saturday, May 1, 2021

Clinical Use of Improved Diagnostic Testing for Detection of Prion Disease

https://prionprp.blogspot.com/2021/05/clinical-use-of-improved-diagnostic.html

***Moreover, sporadic disease has never been observed in breeding colonies or primate research laboratories, most notably among hundreds of animals over several decades of study at the National Institutes of Health25, and in nearly twenty older animals continuously housed in our own facility.***

Even if the prevailing view is that sporadic CJD is due to the spontaneous formation of CJD prions, it remains possible that its apparent sporadic nature may, at least in part, result from our limited capacity to identify an environmental origin.

https://www.nature.com/articles/srep11573

O.05: Transmission of prions to primates after extended silent incubation periods: Implications for BSE and scrapie risk assessment in human populations

Emmanuel Comoy, Jacqueline Mikol, Valerie Durand, Sophie Luccantoni, Evelyne Correia, Nathalie Lescoutra, Capucine Dehen, and Jean-Philippe Deslys Atomic Energy Commission; Fontenay-aux-Roses, France

Prion diseases (PD) are the unique neurodegenerative proteinopathies reputed to be transmissible under field conditions since decades. The transmission of Bovine Spongiform Encephalopathy (BSE) to humans evidenced that an animal PD might be zoonotic under appropriate conditions. Contrarily, in the absence of obvious (epidemiological or experimental) elements supporting a transmission or genetic predispositions, PD, like the other proteinopathies, are reputed to occur spontaneously (atpical animal prion strains, sporadic CJD summing 80% of human prion cases).

Non-human primate models provided the first evidences supporting the transmissibiity of human prion strains and the zoonotic potential of BSE. Among them, cynomolgus macaques brought major information for BSE risk assessment for human health (Chen, 2014), according to their phylogenetic proximity to humans and extended lifetime. We used this model to assess the zoonotic potential of other animal PD from bovine, ovine and cervid origins even after very long silent incubation periods.

*** We recently observed the direct transmission of a natural classical scrapie isolate to macaque after a 10-year silent incubation period,

***with features similar to some reported for human cases of sporadic CJD, albeit requiring fourfold long incubation than BSE. Scrapie, as recently evoked in humanized mice (Cassard, 2014),

***is the third potentially zoonotic PD (with BSE and L-type BSE),

***thus questioning the origin of human sporadic cases.

We will present an updated panorama of our different transmission studies and discuss the implications of such extended incubation periods on risk assessment of animal PD for human health.

===============

***thus questioning the origin of human sporadic cases***

===============

***our findings suggest that possible transmission risk of H-type BSE to sheep and human. Bioassay will be required to determine whether the PMCA products are infectious to these animals.

==============

https://prion2015.files.wordpress.com/2015/05/prion2015abstracts.pdf

***Transmission data also revealed that several scrapie prions propagate in HuPrP-Tg mice with efﬁciency comparable to that of cattle BSE. While the efﬁciency of transmission at primary passage was low, subsequent passages resulted in a highly virulent prion disease in both Met129 and Val129 mice.

***Transmission of the different scrapie isolates in these mice leads to the emergence of prion strain phenotypes that showed similar characteristics to those displayed by MM1 or VV2 sCJD prion.

***These results demonstrate that scrapie prions have a zoonotic potential and raise new questions about the possible link between animal and human prions.

http://www.tandfonline.com/doi/abs/10.1080/19336896.2016.1163048?journalCode=kprn20

PRION 2016 TOKYO

Saturday, April 23, 2016

SCRAPIE WS-01: Prion diseases in animals and zoonotic potential 2016

Prion. 10:S15-S21. 2016 ISSN: 1933-6896 printl 1933-690X online

Taylor & Francis

Prion 2016 Animal Prion Disease Workshop Abstracts

WS-01: Prion diseases in animals and zoonotic potential

Transmission of the different scrapie isolates in these mice leads to the emergence of prion strain phenotypes that showed similar characteristics to those displayed by MM1 or VV2 sCJD prion.

These results demonstrate that scrapie prions have a zoonotic potential and raise new questions about the possible link between animal and human prions.

http://www.tandfonline.com/doi/abs/10.1080/19336896.2016.1163048?journalCode=kprn20

Title: Transmission of scrapie prions to primate after an extended silent incubation period)

*** In complement to the recent demonstration that humanized mice are susceptible to scrapie, we report here the first observation of direct transmission of a natural classical scrapie isolate to a macaque after a 10-year incubation period. Neuropathologic examination revealed all of the features of a prion disease: spongiform change, neuronal loss, and accumulation of PrPres throughout the CNS.

*** This observation strengthens the questioning of the harmlessness of scrapie to humans, at a time when protective measures for human and animal health are being dismantled and reduced as c-BSE is considered controlled and being eradicated.

*** Our results underscore the importance of precautionary and protective measures and the necessity for long-term experimental transmission studies to assess the zoonotic potential of other animal prion strains.

http://www.ars.usda.gov/research/publications/publications.htm?SEQ_NO_115=313160

1: J Infect Dis 1980 Aug;142(2):205-8

Oral transmission of kuru, Creutzfeldt-Jakob disease, and scrapie to nonhuman primates.

Gibbs CJ Jr, Amyx HL, Bacote A, Masters CL, Gajdusek DC.

Kuru and Creutzfeldt-Jakob disease of humans and scrapie disease of sheep and goats were transmitted to squirrel monkeys (Saimiri sciureus) that were exposed to the infectious agents only by their nonforced consumption of known infectious tissues. The asymptomatic incubation period in the one monkey exposed to the virus of kuru was 36 months; that in the two monkeys exposed to the virus of Creutzfeldt-Jakob disease was 23 and 27 months, respectively; and that in the two monkeys exposed to the virus of scrapie was 25 and 32 months, respectively. Careful physical examination of the buccal cavities of all of the monkeys failed to reveal signs or oral lesions. One additional monkey similarly exposed to kuru has remained asymptomatic during the 39 months that it has been under observation.

snip...

The successful transmission of kuru, Creutzfeldt-Jakob disease, and scrapie by natural feeding to squirrel monkeys that we have reported provides further grounds for concern that scrapie-infected meat may occasionally give rise in humans to Creutzfeldt-Jakob disease.

PMID: 6997404

http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=6997404&dopt=Abstract

Recently the question has again been brought up as to whether scrapie is transmissible to man. This has followed reports that the disease has been transmitted to primates. One particularly lurid speculation (Gajdusek 1977) conjectures that the agents of scrapie, kuru, Creutzfeldt-Jakob disease and transmissible encephalopathy of mink are varieties of a single "virus". The U.S. Department of Agriculture concluded that it could "no longer justify or permit scrapie-blood line and scrapie-exposed sheep and goats to be processed for human or animal food at slaughter or rendering plants" (ARC 84/77)" The problem is emphasised by the finding that some strains of scrapie produce lesions identical to the once which characterise the human dementias"

Whether true or not. the hypothesis that these agents might be transmissible to man raises two considerations. First, the safety of laboratory personnel requires prompt attention. Second, action such as the "scorched meat" policy of USDA makes the solution of the scrapie problem urgent if the sheep industry is not to suffer grievously.

snip...

76/10.12/4.6

http://web.archive.org/web/20010305223125/www.bseinquiry.gov.uk/files/yb/1976/10/12004001.pdf

Nature. 1972 Mar 10;236(5341):73-4.

Transmission of scrapie to the cynomolgus monkey (Macaca fascicularis).

Gibbs CJ Jr, Gajdusek DC.

Nature 236, 73 - 74 (10 March 1972); doi:10.1038/236073a0

Transmission of Scrapie to the Cynomolgus Monkey (Macaca fascicularis)

C. J. GIBBS jun. & D. C. GAJDUSEK

National Institute of Neurological Diseases and Stroke, National Institutes of Health, Bethesda, Maryland

SCRAPIE has been transmitted to the cynomolgus, or crab-eating, monkey (Macaca fascicularis) with an incubation period of more than 5 yr from the time of intracerebral inoculation of scrapie-infected mouse brain. The animal developed a chronic central nervous system degeneration, with ataxia, tremor and myoclonus with associated severe scrapie-like pathology of intensive astroglial hypertrophy and proliferation, neuronal vacuolation and status spongiosus of grey matter. The strain of scrapie virus used was the eighth passage in Swiss mice (NIH) of a Compton strain of scrapie obtained as ninth intracerebral passage of the agent in goat brain, from Dr R. L. Chandler (ARC, Compton, Berkshire).

http://www.nature.com/nature/journal/v236/n5341/abs/236073a0.html

http://scrapie-usa.blogspot.com/2010/04/scrapie-and-atypical-scrapie.html

Wednesday, February 16, 2011

IN CONFIDENCE

SCRAPIE TRANSMISSION TO CHIMPANZEES

IN CONFIDENCE

reference...

RB3.20

TRANSMISSION TO CHIMPANZEES

1. Kuru and CJD have been successfully transmitted to chimpanzees but scrapie and TME have not.

2. We cannot say that scrapie will not transmit to chimpanzees. There are several scrapie strains and I am not aware that all have been tried (that would have to be from mouse passaged material). Nor has a wide enough range of field isolates subsequently strain typed in mice been inoculated by the appropriate routes (i/c, ilp and i/v) :

3. I believe the proposed experiment to determine transmissibility, if conducted, would only show the susceptibility or resistance of the chimpanzee to infection/disease by the routes used and the result could not be interpreted for the predictability of the susceptibility for man. Proposals for prolonged oral exposure of chimpanzees to milk from cattle were suggested a long while ago and rejected.

4. In view of Dr Gibbs' probable use of chimpazees Mr Wells' comments (enclosed) are pertinent. I have yet to receive a direct communication from Dr Schellekers but before any collaboration or provision of material we should identify the Gibbs' proposals and objectives.

5. A positive result from a chimpanzee challenged severely would likely create alarm in some circles even if the result could not be interpreted for man. I have a view that all these agents could be transmitted provided a large enough dose by appropriate routes was given and the animals kept long enough. Until the mechanisms of the species barrier are more clearly understood it might be best to retain that hypothesis.

6. A negative result would take a lifetime to determine but that would be a shorter period than might be available for human exposure and it would still not answer the question regarding mans' susceptibility. In the meantime no doubt the negativity would be used defensively. It would however be counterproductive if the experiment finally became positive. We may learn more about public reactions following next Monday' s meeting.

R. Bradley

23 September 1990

CVO (+Mr Wells' comments)

Dr T W A Little

Dr B J Shreeve

90/9.23/1.1.

http://web.archive.org/web/20090506041740/http://www.bseinquiry.gov.uk/files/yb/1990/09/23001001.pdf

IN CONFIDENCE CHIMPANZEES

CODE 18-77 Reference RB3.46

Some further information that may assist in decision making has been gained by discussion with Dr Rosalind Ridley.

She says that careful study of Gajdusek's work shows no increased susceptibility of chimpanzees over New World Monkeys such as Squirrel Monkeys. She does not think it would tell you anything about the susceptibility to man. Also Gajdusek did not, she believes, challenge chimpanzees with scrapie as severely as we did pigs and we know little of that source of scrapie. Comparisons would be difficult. She also would not expect the Home Office to sanction such experiments here unless there was a very clear and important objective that would be important for human health protection. She doubted such a case could be made. If this is the case she thought it would be unethical to do an experiment abroad because we could not do it in our own country.

Retrospectively she feels they should have put up more marmosets than they did. They all remain healthy. They would normally regard the transmission as negative if no disease resulted in five years.

We are not being asked for a decision but I think that before we made one we should gain as much knowledge as we can. If we decided to proceed we would have to bear any criticisms for many years if there was an adverse view by scientists ormedia. This should not be undertaken lightly. There is already some adverse comment here, I gather, on the pig experiment though that will subside.

The Gibbs' (as' distinct from Schellekers') study is somewhat different. We are merely supplying material for comparative studies in a laboratory with the greatest experience of human SEs in the world and it has been sanctioned by USDA (though we do not know for certain yet if chimpanzees specifically will be used). This would keep it at a lower profile than if we conducted such an experiment in the UK or Europe.

I consider we must have very powerful and defendable objectives to go beyond Gibbs' proposed experiments and should not initiate others just because an offer has been made.

Scientists have a responsibility to seek other methods of investigative research other than animal experimentation. At present no objective has convinced me we need to do research using Chimpanzees - a species in need of protection. Resisting such proposals would enable us to communicate that information to the scientist and the public should the need arise. A line would have been drawn.

CVO cc Dr T Dr B W A Little Dr B J Shreeve

R Bradley

26 September 1990

90/9.26/3.2

http://web.archive.org/web/20090506041605/http://www.bseinquiry.gov.uk/files/yb/1990/09/26003001.pdf

this is tse prion political theater here, i.e. what i call TSE PRION POKER...tss

http://web.archive.org/web/20031028205208/www.bseinquiry.gov.uk/files/yb/1990/08/28002001.pdf

http://web.archive.org/web/20030822170317/www.bseinquiry.gov.uk/files/yb/1990/11/01005001.pdf

3. Prof. A. Robertson gave a brief account of BSE. The US approach was to accord it a very low profile indeed. Dr. A Thiermann showed the picture in the ''Independent'' with cattle being incinerated and thought this was a fanatical incident to be avoided in the US at all costs.

snip...

PAGE 26

Transmission Studies

The occurrence of CWD must be viewed against the contest of the locations in which it occurred. It was an incidental and unwelcome complication of the respective wildlife research programmes. Despite its subsequent recognition as a new disease of cervids, therefore justifying direct investigation, no specific research funding was forthcoming. The USDA veiwed it as a wildlife problem and consequently not their province! ...page 26.

snip...see;

IN CONFIDENCE

PERCEPTIONS OF UNCONVENTIONAL SLOW VIRUS DISEASE OF ANIMALS IN THE USA

GAH WELLS

REPORT OF A VISIT TO THE USA

APRIL-MAY 1989

http://web.archive.org/web/20090506002237/http://www.bseinquiry.gov.uk/files/mb/m11b/tab01.pdf

Thursday, July 29, 2021

TSE PRION OCCUPATIONAL EXPOSURE VIA ANIMAL OR HUMAN, iatrogenic transmission, nvCJD or sCJD, what if?

https://itseprion.blogspot.com/2021/07/tse-prion-occupational-exposure-via.html

Sunday, January 10, 2021

APHIS Concurrence With OIE Risk Designation for Bovine Spongiform Encephalopathy [Docket No. APHIS-2018-0087] Singeltary Submission June 17, 2019

APHIS Concurrence With OIE Risk Designation for Bovine Spongiform Encephalopathy [Docket No. APHIS-2018-0087] Singeltary Submission

Greetings APHIS et al,

I would kindly like to comment on APHIS Concurrence With OIE Risk Designation for Bovine Spongiform Encephalopathy [Docket No. APHIS-2018-0087], and my comments are as follows, with the latest peer review and transmission studies as references of evidence.

THE OIE/USDA BSE Minimal Risk Region MRR is nothing more than free pass to import and export the Transmissible Spongiform Encephalopathy TSE Prion disease. December 2003, when the USDA et al lost it's supposedly 'GOLD CARD' ie BSE FREE STATUS (that was based on nothing more than not looking and not finding BSE), once the USA lost it's gold card BSE Free status, the USDA OIE et al worked hard and fast to change the BSE Geographical Risk Statuses i.e. the BSE GBR's, and replaced it with the BSE MRR policy, the legal tool to trade mad cow type disease TSE Prion Globally. The USA is doing just what the UK did, when they shipped mad cow disease around the world, except with the BSE MRR policy, it's now legal.

Also, the whole concept of the BSE MRR policy is based on a false pretense, that atypical BSE is not transmissible, and that only typical c-BSE is transmissible via feed. This notion that atypical BSE TSE Prion is an old age cow disease that is not infectious is absolutely false, there is NO science to show this, and on the contrary, we now know that atypical BSE will transmit by ORAL ROUTES, but even much more concerning now, recent science has shown that Chronic Wasting Disease CWD TSE Prion in deer and elk which is rampant with no stopping is sight in the USA, and Scrapie TSE Prion in sheep and goat, will transmit to PIGS by oral routes, this is our worst nightmare, showing even more risk factors for the USA FDA PART 589 TSE PRION FEED ban.

The FDA PART 589 TSE PRION FEED ban has failed terribly bad, and is still failing, since August 1997. there is tonnage and tonnage of banned potential mad cow feed that went into commerce, and still is, with one decade, 10 YEARS, post August 1997 FDA PART 589 TSE PRION FEED ban, 2007, with 10,000,000 POUNDS, with REASON, Products manufactured from bulk feed containing blood meal that was cross contaminated with prohibited meat and bone meal and the labeling did not bear cautionary BSE statement. you can see all these feed ban warning letters and tonnage of mad cow feed in commerce, year after year, that is not accessible on the internet anymore like it use to be, you can see history of the FDA failure August 1997 FDA PART 589 TSE PRION FEED ban here, but remember this, we have a new outbreak of TSE Prion disease in a new livestock species, the camel, and this too is very worrisome.

WITH the OIE and the USDA et al weakening the global TSE prion surveillance, by not classifying the atypical Scrapie as TSE Prion disease, and the notion that they want to do the same thing with typical scrapie and atypical BSE, it's just not scientific.

WE MUST abolish the BSE MRR policy, go back to the BSE GBR risk assessments by country, and enhance them to include all strains of TSE Prion disease in all species. With Chronic Wasting CWD TSE Prion disease spreading in Europe, now including, Norway, Finland, Sweden, also in Korea, Canada and the USA, and the TSE Prion in Camels, the fact the the USA is feeding potentially CWD, Scrapie, BSE, typical and atypical, to other animals, and shipping both this feed and or live animals or even grains around the globe, potentially exposed or infected with the TSE Prion. this APHIS Concurrence With OIE Risk Designation for Bovine Spongiform Encephalopathy [Docket No. APHIS-2018-0087], under it's present definition, does NOT show the true risk of the TSE Prion in any country. as i said, it's nothing more than a legal tool to trade the TSE Prion around the globe, nothing but ink on paper.

AS long as the BSE MRR policy stays in effect, TSE Prion disease will continued to be bought and sold as food for both humans and animals around the globe, and the future ramifications from friendly fire there from, i.e. iatrogenic exposure and transmission there from from all of the above, should not be underestimated. ...

https://beta.regulations.gov/document/APHIS-2018-0087-0002

https://downloads.regulations.gov/APHIS-2018-0087-0002/attachment_1.pdf

https://bovineprp.blogspot.com/2019/06/aphis-concurrence-with-oie-risk.html

WEDNESDAY, MARCH 24, 2021

USDA Animal and Plant Health Inspection Service 2020 IMPACT REPORT BSE TSE Prion Testing and Surveillance MIA

https://animalhealthreportpriontse.blogspot.com/2021/03/usda-animal-and-plant-health-inspection.html

WEDNESDAY, DECEMBER 2, 2020

EFSA Evaluation of public and animal health risks in case of a delayed post-mortem inspection in ungulates EFSA Panel on Biological Hazards (BIOHAZ) ADOPTED: 21 October 2020

i wonder if a 7 month delay on a suspect BSE case in Texas is too long, on a 48 hour turnaround, asking for a friend???

https://efsaopinionbseanimalprotein.blogspot.com/2020/12/efsa-evaluation-of-public-and-animal.html

MONDAY, NOVEMBER 30, 2020

***> REPORT OF THE MEETING OF THE OIE SCIENTIFIC COMMISSION FOR ANIMAL DISEASES Paris, 9–13 September 2019 BSE, TSE, PRION

see updated concerns with atypical BSE from feed and zoonosis...terry

https://animalhealthreportpriontse.blogspot.com/2020/11/report-of-meeting-of-oie-scientific.html

WEDNESDAY, DECEMBER 23, 2020

BSE research project final report 2005 to 2008 SE1796 SID5

http://bovineprp.blogspot.com/2020/12/bse-research-project-final-report-2005.html

***> Wednesday, January 23, 2019

***> CFIA SFCR Guidance on Specified risk material (SRM) came into force on January 15, 2019 <***

https://specifiedriskmaterial.blogspot.com/2019/01/cfia-sfcr-guidance-on-specified-risk.html

TUESDAY, JANUARY 5, 2021

Exploration of genetic factors resulting in abnormal disease in cattle experimentally challenged with bovine spongiform encephalopathy

https://bovineprp.blogspot.com/2021/01/exploration-of-genetic-factors.html

*** Singeltary reply ; Molecular, Biochemical and Genetic Characteristics of BSE in Canada Singeltary reply ;

https://journals.plos.org/plosone/article/comment?id=10.1371/annotation/4f9be886-69fe-4c7c-922b-85b0ecbe6d53

IBNC Tauopathy or TSE Prion disease, it appears, no one is sure

Terry S. Singeltary Sr., 03 Jul 2015 at 16:53 GMT

PLOS ONE Journal

IBNC Tauopathy or TSE Prion disease, it appears, no one is sure

Terry S. Singeltary Sr., 03 Jul 2015 at 16:53 GMT

***however in 1 C-type challenged animal, Prion 2015 Poster Abstracts S67 PrPsc was not detected using rapid tests for BSE.

***Subsequent testing resulted in the detection of pathologic lesion in unusual brain location and PrPsc detection by PMCA only.

*** IBNC Tauopathy or TSE Prion disease, it appears, no one is sure ***

https://journals.plos.org/plosone/article/comment?id=10.1371/annotation/5adef4ac-a7e4-46a4-8806-c8533d5c862c

THURSDAY, AUGUST 19, 2021

TME to cattle equal atypical L-type BSE USA, madcow origin, what if?

https://transmissiblespongiformencephalopathy.blogspot.com/2021/08/tme-to-cattle-equal-atypical-l-type-bse.html

MAD CAMEL DISEASE OR CPD CAMEL PRION DISEASE

Tuesday, April 27, 2021

Working Document on Camel Prion Disease (CPrD) 14/09/2020

https://camelusprp.blogspot.com/2021/04/working-document-on-camel-prion-disease.html

Owens, Julie

From: Terry S. Singeltary Sr. [flounder9@verizon.net]

Sent: Monday, July 24, 2006 1:09 PM

To: FSIS Regulations Comments

Subject: [Docket No. FSIS-2006-0011] FSIS Harvard Risk Assessment of Bovine Spongiform Encephalopathy (BSE)

Page 1 of 98

8/3/2006

Greetings FSIS,

I would kindly like to comment on the following ;

https://citeseerx.ist.psu.edu/viewdoc/download?doi=10.1.1.230.8886&rep=rep1&type=pdf

MONDAY, NOVEMBER 16, 2015

Docket No. APHIS-2007-0127 Scrapie in Sheep and Goats Terry Singeltary Sr. Submission

Docket No. APHIS-2007-0127 Scrapie in Sheep and Goats Terry Singeltary Sr. Submission Docket No. APHIS-2007-0127

Scrapie in Sheep and Goats SUMMARY: We are reopening the comment period for our proposed rule that would revise completely the scrapie regulations, which concern the risk groups and categories established for individual animals and for flocks, the use of genetic testing as a means of assigning risk levels to animals, movement restrictions for animals found to be genetically less susceptible or resistant to scrapie, and record keeping requirements. This action will allow interested persons additional time to prepare and submit comments. DATES: The comment period for the proposed rule published on September 10, 2015 (80 FR 54660-54692) is reopened. We will consider all comments that we receive on or before December 9, 2015. ...

http://www.regulations.gov/#!documentDetail;D=APHIS-2007-0127-0001

http://www.gpo.gov/fdsys/pkg/FR-2015-11-16/html/2015-29179.htm

http://www.regulations.gov/#!docketDetail;D=APHIS-2007-0127

COMMENT SUBMISSION TERRY S. SINGELTARY SR.

WITH regards to Docket No. APHIS-2007-0127 Scrapie in Sheep and Goats, I kindly submit the following ;

>>>The last major revision of the scrapie regulations occurred on August 21, 2001, when we published in the Federal Register(66 FR 43964, Docket No. 97-093-5) a final rule amending part 79 by imposing additional restrictions on the interstate movement of sheep and goats.<<<

Indeed, much science has changed about the Scrapie TSE prion, including more science linking Scrapie to humans. sadly, politics, industry, and trade, have not changed, and those usually trump sound science, as is the case with all Transmissible Spongiform Encephalopathy TSE Prion disease in livestock producing animals and the OIE. we can look no further at the legal trading of the Scrapie TSE prion both typical and atypical of all strains, and CWD all stains. With as much science of old, and now more new science to back this up, Scrapie of all types i.e. atypical and typical, BSE all strains, and CWD all strains, should be regulated in trade as BSE TSE PRION. In fact, I urge APHIS et al and the OIE, and all trading partners to take heed to the latest science on the TSE prion disease, all of them, and seriously reconsider the blatant disregards for human and animal health, all in the name of trade, with the continued relaxing of TSE Prion trade regulations through the ‘NEGLIGIBLE BSE RISK’ PROGRAM, which was set up to fail in the first place. If the world does not go back to the ‘BSE RISK ASSESSMENTS’, enhance, and or change that assessment process to include all TSE prion disease, i.e. ‘TSE RISK ASSESSMENT’, if we do not do this and if we continue this farce with OIE and the USDA et al, and the ‘NEGLIGIBLE BSE RISK’ PROGRAM, we will never eradicate the TSE prion aka mad cow type disease, they will continue to mutate and spread among species of human and animal origin, and they will continue to kill. ... please see ;

O.05: Transmission of prions to primates after extended silent incubation periods: Implications for BSE and scrapie risk assessment in human populations

see my full Docket Submission here;

https://scrapie-usa.blogspot.com/2015/11/docket-no-aphis-2007-0127-scrapie-in.html

JOHN CORNYN TEXAS UNITED STATES SENATE WASHINGTON, DC 20510-4305 April 26,2005

Mr. Terry Singeltary

P.O. Box

Bacliff, Texas 77518

Dear Mr. Singeltary:

In response to your recent request for my assistance, I have contacted the National Institutes of Health. I will write you again as soon as I receive a reply. I appreciate having the opportunity to represent you in the United States Senate and to be of service in this matter.

Sincerely,

JOHN CORNYN United States Senator JC:djl

===============

JOHN CORNYN TEXAS UNITED STATES SENATE WASHINGTON, DC 20510-4305

May 18,2005

Mr. Terry Singeltary

P.O. Box

Bacliff, Texas 77518

Dear Mr. Singeltary:

Enclosed is the reply I received from the Department of Health and Human Services in response to my earlier inquiry on your behalf. I hope this will be useful to you. I appreciate having the opportunity to represent you in the United States Senate. Thank you for taking time to contact me. Sincerely,

JOHN CORNYN United States Senate JC:djl Enclosure

DEPARTMENT OF HEALTH & HUMAN SERVICES National Institutes of Health National Institute of Neurological Disorders and Stroke NINDS Building 31, Room 8A52 31 Center Dr., MSC 2540 Bethesda, Maryland 20892-2540 Phone: 301-496-9746 Fax: 301-496-0296 Email: [log in to unmask]

May 10, 2005

The Honorable John Cornyn United States Senator Occidental Tower5005 LBJ Freeway, Suite 1150Dallas, Texas 75244-6199

Dear Senator Cornyn:

Your letter to the National Institutes of Health (NIH) forwarding correspondence from Mr. Terry S. Singeltary, Sr., has been forwarded to me for reply. Mr. Singeltary is concerned about the preservation of Creutzfeldt-Jakob disease (CJD) brain samples that have been maintained by the National Institute of Neurological Disorders and Stroke (NINDS) Intramural Research program for many years. I am sorry to learn that Mr. Singeltary's mother died of CJD and can certainly understand his desire that any tissues that could help investigators unravel the puzzle of this deadly disease are preserved. I hope he will be pleased to learn that all the brains and other tissues with potential to help scientists learn about CJD are, and will continue to be, conserved. (The tissues that are discarded are those that have either decayed to an extent that renders them no longer appropriate for research or those for which we do not have sufficient identification.) The purpose of gathering these brains and tissues is to help scientists learn about CJD. To that end, some of the NINDS-held samples are distributed to investigators who can demonstrate that they have a compelling research or public health need for such materials. For example, samples have been transferred to NIH grantee Dr. Pierluigi Gambetti, who heads the National Prion Diseases Pathology Surveillance Center at Case Western Reserve University in Ohio and works with the Centers for Disease Control and Prevention to monitor all cases of CJD in the United States. Dr. Gambetti studies the tissues to learn about the formation, physical and chemical properties, and pathogenic mechanisms of prion proteins, which are believed to be involved inthe cause of CJD. Samples have also been transferred to Dr. David Asher, at the U.S. Food and Drug Administration, for use in assessing a potential diagnostic test for CJD.

Page 2 - The Honorable John Cornyn

in closing, we know that donating organs and tissue from loved ones is a very difficult and personal choice that must often be made at the most stressful of times. We at the NINDS are grateful to those stalwart family members who make this choice in the selfless hope that it will help others afflicted with CJD. We also know the invaluable contribution such donations make to the advancement of medical science, and we are dedicated to the preservation of all of the tissue samples that can help in our efforts to overcome CJD.

I hope this information is helpful to you in responding to Mr. Singeltary. Sincerely,

Story C. Landis, Ph.D. Director, National Institute of Neurological Disorders and Stroke

snip...see full text;

http://creutzfeldt-jakob-disease.blogspot.com/2009/08/cjdstraight-talk-withjames.html

https://www.upi.com/Science_News/2005/05/31/NIH-says-it-will-preserve-CJD-brains/67711117574761/

Diagnosis and Reporting of Creutzfeldt-Jakob Disease Singeltary, Sr et al.

JAMA.2001; 285: 733-734. Vol. 285 No. 6, February 14, 2001 JAMA

Diagnosis and Reporting of Creutzfeldt-Jakob Disease

To the Editor: In their Research Letter, Dr Gibbons and colleagues1 reported that the annual US death rate due to Creutzfeldt-Jakob disease (CJD) has been stable since 1985. These estimates, however, are based only on reported cases, and do not include misdiagnosed or preclinical cases. It seems to me that misdiagnosis alone would drastically change these figures. An unknown number of persons with a diagnosis of Alzheimer disease in fact may have CJD, although only a small number of these patients receive the postmortem examination necessary to make this diagnosis. Furthermore, only a few states have made CJD reportable. Human and animal transmissible spongiform encephalopathies should be reportable nationwide and internationally..

Terry S. Singeltary, Sr Bacliff, Tex 1. Gibbons RV, Holman RC, Belay ED, Schonberger LB. Creutzfeldt-Jakob disease in the United States: 1979-1998. JAMA. 2000;284:2322-2323.

http://jama.jamanetwork.com/article.aspx?articleid=1031186

Tracking spongiform encephalopathies in North America

Xavier Bosch

Available online 29 July 2003.

Volume 3, Issue 8, August 2003, Page 463

“My name is Terry S Singeltary Sr, and I live in Bacliff, Texas. I lost my mom to hvCJD (Heidenhain variant CJD) and have been searching for answers ever since. What I have found is that we have not been told the truth. CWD in deer and elk is a small portion of a much bigger problem..”

http://www.thelancet.com/journals/laninf/article/PIIS1473309903007151/fulltext

http://download.thelancet..com/pdfs/journals/1473-3099/PIIS1473309903007151.pdf

January 28, 2003; 60 (2) VIEWS & REVIEWS

RE-Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob disease in the United States Terry S. Singeltary, retired (medically)

Published March 26, 2003

26 March 2003

Terry S. Singeltary, retired (medically) CJD WATCH

I lost my mother to hvCJD (Heidenhain Variant CJD). I would like to comment on the CDC's attempts to monitor the occurrence of emerging forms of CJD. Asante, Collinge et al [1] have reported that BSE transmission to the 129-methionine genotype can lead to an alternate phenotype that is indistinguishable from type 2 PrPSc, the commonest sporadic CJD. However, CJD and all human TSEs are not reportable nationally. CJD and all human TSEs must be made reportable in every state and internationally. I hope that the CDC does not continue to expect us to still believe that the 85%+ of all CJD cases which are sporadic are all spontaneous, without route/source. We have many TSEs in the USA in both animal and man. CWD in deer/elk is spreading rapidly and CWD does transmit to mink, ferret, cattle, and squirrel monkey by intracerebral inoculation. With the known incubation periods in other TSEs, oral transmission studies of CWD may take much longer. Every victim/family of CJD/TSEs should be asked about route and source of this agent. To prolong this will only spread the agent and needlessly expose others. In light of the findings of Asante and Collinge et al, there should be drastic measures to safeguard the medical and surgical arena from sporadic CJDs and all human TSEs. I only ponder how many sporadic CJDs in the USA are type 2 PrPSc?

http://n.neurology.org/content/re-monitoring-occurrence-emerging-forms-creutzfeldt-jakob-disease-united-states

SPORADIC CJD LAYING ODDS

https://link.springer.com/chapter/10.1007/0-387-21755-X_14

In brief

BMJ 2000; 320 doi: https://doi.org/10.1136/bmj.320.7226.8/b (Published 01 January 2000)

Cite this as: BMJ 2000;320:8

Rapid Response:

02 January 2000

Terry S Singeltary

retired

U.S. Scientist should be concerned with a CJD epidemic in the U.S., as well... In reading your short article about 'Scientist warn of CJD epidemic' news in brief Jan. 1, 2000. I find the findings in the PNAS old news, made famous again. Why is the U.S. still sitting on their butts, ignoring the facts? We have the beginning of a CJD epidemic in the U.S., and the U.S. Gov. is doing everything in it's power to conceal it.

The exact same recipe for B.S.E. existed in the U.S. for years and years. In reading over the Qualitative Analysis of BSE Risk Factors-1, this is a 25 page report by the USDA:APHIS:VS. It could have been done in one page. The first page, fourth paragraph says it all;

"Similarities exist in the two countries usage of continuous rendering technology and the lack of usage of solvents, however, large differences still remain with other risk factors which greatly reduce the potential risk at the national level."

Then, the next 24 pages tries to down-play the high risks of B.S.E. in the U.S., with nothing more than the cattle to sheep ratio count, and the geographical locations of herds and flocks. That's all the evidence they can come up with, in the next 24 pages.

Something else I find odd, page 16;

"In the United Kingdom there is much concern for a specific continuous rendering technology which uses lower temperatures and accounts for 25 percent of total output. This technology was _originally_ designed and imported from the United States. However, the specific application in the production process is _believed_ to be different in the two countries."

A few more factors to consider, page 15;

"Figure 26 compares animal protein production for the two countries. The calculations are based on slaughter numbers, fallen stock estimates, and product yield coefficients. This approach is used due to variation of up to 80 percent from different reported sources. At 3.6 million tons, the United States produces 8 times more animal rendered product than the United Kingdom."

"The risk of introducing the BSE agent through sheep meat and bone meal is more acute in both relative and absolute terms in the United Kingdom (Figures 27 and 28). Note that sheep meat and bone meal accounts for 14 percent, or 61 thousand tons, in the United Kingdom versus 0.6 percent or 22 thousand tons in the United States. For sheep greater than 1 year, this is less than one-tenth of one percent of the United States supply."

"The potential risk of amplification of the BSE agent through cattle meat and bone meal is much greater in the United States where it accounts for 59 percent of total product or almost 5 times more than the total amount of rendered product in the United Kingdom."

Considering, it would only take _one_ scrapie infected sheep to contaminate the feed. Considering Scrapie has run rampant in the U.S. for years, as of Aug. 1999, 950 scrapie infected flocks. Also, Considering only one quarter spoonful of scrapie infected material is lethal to a cow.

Considering all this, the sheep to cow ration is meaningless. As I said, it's 24 pages of B.S.e.

To be continued...

Terry S. Singeltary Sr.

Bacliff, Texas USA

Competing interests: No competing interests

https://www.bmj.com/rapid-response/2011/10/28/us-scientist-should-be-concerned-cjd-epidemic-us-well

Rapid response to:

US scientists develop a possible test for BSE

15 November 1999

Terry S Singeltary

BMJ 1999; 319 doi: https://doi.org/10.1136/bmj.319.7220.1312b (Published 13 November 1999)

Cite this as: BMJ 1999;319:1312

Article Related content Article metrics

Rapid responses

Response Rapid Response: Re: vCJD in the USA * BSE in U.S. In reading the recent article in the BMJ about the potential BSE tests being developed in the U.S. and Bart Van Everbroeck reply. It does not surprize me, that the U.S. has been concealing vCJD. There have been people dying from CJD, with all the symptoms and pathological findings that resemble U.K. vCJD for some time. It just seems that when there is one found, they seem to change the clarical classification of the disease, to fit their agenda. I have several autopsies, stating kuru type amyloid plaques, one of the victims was 41 years of age. Also, my Mom died a most hideous death, Heidenhain Variant Creutzfeldt Jakob disease. Her symptoms resemble that of all the U.K. vCJD victims. She would jerk so bad at times, it would take 3 of us to hold her down, while she screamed "God, what's wrong with me, why can't I stop this." 1st of symptoms to death, 10 weeks, she went blind in the first few weeks. But, then they told me that this was just another strain of sporadic CJD. They can call it what ever they want, but I know what I saw, and what she went through. Sporadic, simply means, they do not know. My neighbors Mom also died from CJD. She had been taking a nutritional supplement which contained the following; vacuum dried bovine BRAIN, bone meal, bovine EYE, veal bone, bovine liver powder, bovine adrenal, vacuum dried bovine kidney, and vacuum dried porcine stomach. As I said, this woman taking these nutritional supplements, died from CJD. The particular batch of pills that was located, in which she was taking, was tested. From what I have heard, they came up negative, for the prion protein. But, in the same breath, they said their testing, may not have been strong enough to pick up the infectivity. Plus, she had been taking these type pills for years, so, could it have come from another batch?

CWD is just a small piece of a very big puzzle. I have seen while deer hunting, deer, squirrels and birds, eating from cattle feed troughs where they feed cattle, the high protein cattle by products, at least up until Aug. 4, 1997.

So why would it be so hard to believe that this is how they might become infected with a TSE. Or, even by potentially infected land. It's been well documented that it could be possible, from scrapie. Cats becoming infected with a TSE. Have you ever read the ingredients on the labels of cat and dog food? But, they do not put these tissues from these animals in pharmaceuticals, cosmetics, nutritional supplements, hGH, hPG, blood products, heart valves, and the many more products that come from bovine, ovine, or porcine tissues and organs. So, as I said, this CWD would be a small piece of a very big puzzle. But, it is here, and it most likely has killed. You see, greed is what caused this catastrophe, rendering and feeding practices. But, once Pandora's box was opened, the potential routes of infection became endless.

No BSE in the U.S.A.? I would not be so sure of that considering that since 1990;

Since 1990 the U.S. has raised 1,250,880,700 cattle;

Since 1990 the U.S. has ONLY checked 8,881 cattle brains for BSE, as of Oct. 4, 1999;

There are apprx. 100,000 DOWNER cattle annually in the U.S., that up until Aug. 4, 1997 went to the renders for feed;

Scrapie running rampant for years in the U.S., 950 infected FLOCKS, as of Aug. 1999;

Our feeding and rendering practices have mirrored that of the U.K. for years, some say it was worse. Everything from the downer cattle, to those scrapie infected sheep, to any roadkill, including the city police horse and the circus elephant went to the renders for feed and other products for consumption. Then they only implemented a partial feed ban on Aug. 4, 1997, but pigs, chickens, dogs, and cats, and humans were exempt from that ban. So they can still feed pigs and chickens those potentially TSE tainted by-products, and then they can still feed those by-products back to the cows. I believe it was Dr. Joe Gibbs, that said, the prion protein, can survive the digestinal track. So you have stopped nothing. It was proven in Oprah Winfrey's trial, that Cactus Cattle feeders, sent neurologically ill cattle, some with encephalopathy stamped on the dead slips, were picked up and sent to the renders, along with sheep carcasses. Speaking of autopsies, I have a stack of them, from CJD victims. You would be surprised of the number of them, who ate cow brains, elk brains, deer brains, or hog brains.

I believe all these TSE's are going to be related, and originally caused by the same greedy Industries, and they will be many. Not just the Renders, but you now see, that they are re-using medical devices that were meant for disposal. Some medical institutions do not follow proper auto- claving procedures (even Olympus has put out a medical warning on their endescopes about CJD, and the fact you cannot properly clean these instruments from TSE's), and this is just one product. Another route of infection.

Regardless what the Federal Government in the U.S. says. It's here, I have seen it, and the longer they keep sweeping it under the rug and denying the fact that we have a serious problem, one that could surpass aids (not now, but in the years to come, due to the incubation period), they will be responsible for the continued spreading of this deadly disease.

It's their move, it's CHECK, but once CHECKMATE has been called, how many thousands or millions, will be at risk or infected or even dead. You can't play around with these TSE's. I cannot stress that enough. They are only looking at body bags, and the fact the count is so low. But, then you have to look at the fact it is not a reportable disease in most states, mis-diagnosis, no autopsies performed. The fact that their one-in-a- million theory is a crude survey done about 5 years ago, that's a joke, under the above circumstances. A bad joke indeed........

The truth will come, but how many more have to die such a hideous death. It's the Government's call, and they need to make a serious move, soon. This problem, potential epidemic, is not going away, by itself.

Terry S. Singeltary Sr.

Bacliff, Texas 77518 USA

flounder@wt.net

Competing interests: No competing interests

https://www.bmj.com/content/319/7220/1312.3

TUESDAY, AUGUST 03, 2021

USA Tables of Cases Examined National Prion Disease Pathology Surveillance Center Cases Examined July 9th, 2021

https://creutzfeldt-jakob-disease.blogspot.com/2021/08/usa-tables-of-cases-examined-national.html

Wednesday, July 28, 2021

France issues moratorium on prion research after fatal brain disease strikes two lab workers

https://itseprion.blogspot.com/2021/07/france-issues-moratorium-on-prion.html

Thursday, July 29, 2021

TSE PRION OCCUPATIONAL EXPOSURE VIA ANIMAL OR HUMAN, iatrogenic transmission, nvCJD or sCJD, what if?

https://itseprion.blogspot.com/2021/07/tse-prion-occupational-exposure-via.html

Wednesday, June 30, 2021

Flexible Bronchoscopes and Updated Recommendations for Reprocessing: FDA Safety Communication (Prion Disease) Date Issued: June 25, 2021

https://itseprion.blogspot.com/2021/06/flexible-bronchoscopes-and-updated.html

Case Reports

A Unique Presentation of Creutzfeldt-Jakob Disease in a Patient Consuming Deer Antler Velvet

*Joseph Wiedemer, *Yanely Sanchez Ceja, An Cao and Ibrahim Mustafa Department of Neurology, Wright State University Boonshoft School of Medicine, USA Article history

Received: 17-02-2021 Revised: 08-03-2021 Accepted: 13-03-2021

Corresponding Author: Joseph Wiedemer Department of Neurology, Wright State University Boonshoft School of Medicine, USA

Email: Wiedemer.2@wright.edu

*These authors contributed equally to this work and are considered to be co-first authors

Abstract: Creutzfeldt-Jakob Disease (CJD) is a rare rapidly progressive neurodegenerative prion disorder that is usually sporadic but may also be acquired from exposure to infected sources, classically via infections of bovine or human etiology. Cervid transmission of CJD is of particular concern in North America given the rapid spread of Chronic Wasting Disease (CWD)- the Cervid version of CJD. We present a 61-year-old male patient admitted to our service with a one month history of progressive confusion and gait instability, which led to an initial suspicion of Corticobasal Syndrome (CBS) with unusually rapid progression. CJD was also suspected upon learning that the patient began taking deer antler velvet and bovine colostrum supplements roughly two months prior. The diagnosis of CJD was subsequently confirmed by MRI and RT-QuIC CSF assay. Providers should consider Creutzfeldt-Jakob Disease in the differential diagnosis of a patient with cervid exposure and/or in patients with a presentation resembling corticobasal degeneration, especially if symptom onset is rapid. Although it is unclear how this patient acquired CJD, the potential for prion transmission from cervids to humans should be further investigated by the academic community with considerable urgency.

Introduction

Creutzfeldt-Jakob Disease (CJD) is a rare, rapidly progressive neurodegenerative prion disorder that is inevitably fatal within a year of diagnosis (Iwasaki, 2017). In the US, 5,212 cases of prion disease demonstrated a median age of death of 67 years, an even gender distribution and an increased annual incidence among those >65 years of age between 2003-2015 (Maddox et al., 2020). CJD most-commonly presents in a sporadic form, but is observed in ~15% of cases as acquired variant-CJD (vCJD), in which prions are transmitted to humans from bovine, cannibalistic, or iatrogenic origins (Iwasaki, 2017; Maddox et al., 2020). Acquired prion disease in humans is also known as Transmissible Spongiform Encephalopathy (TSE) and is most famously exemplified by the bovine-derived TSE outbreak in the United Kingdom-commonly referred to as “mad cow disease”-which occurred in 1986 and peaked in 1993 (Casalone and Hope, 2018).

Prion disease of elk and deer is known as Chronic Wasting Disease (CWD) and is also known to be inevitably fatal (USGS National Wildlife Health Center, 2020; Hannaoui et al., 2017). Unlike human prion disease, CWD is highly contagious among cervids and has been expeditiously spreading throughout North America (USGS National Wildlife Health Center, 2020; Hannaoui et al., 2017; Waddell et al., 2018; Moreno and Telling, 2017). Furthermore, in contrast to CJD, prion disease in cervids typically has a subtle clinical presentation, which makes diagnosis and ecological analyses extremely difficult (Hannaoui et al., 2017; Moreno and Telling, 2017). In the United States, documented cases of CWD have spread from 2 to 26 states since 2000 (USGS National Wildlife Health Center, 2020; Osterholm et al., 2019). Attempts to develop an oral vaccine for livestock against CWD have shown recent promise, but the risk of transmission to humans remains poorly understood (Taschuk et al., 2017). A statewide epidemiological study in Colorado in 2006 was equivocal in discerning an increased risk of CJD in areas heavily impacted by CWD between 1979-2001 (RR 0.81, CI 0.40-1.63) (MaWhinney et al., 2006). Such studies have been unable to determine the risk of prion transmission by cervids largely due to the rare frequency of CJD, diagnostic difficulties and variability of symptom onset following exposure (MaWhinney et al., 2006; Nemani et al., 2020).

Although there are no confirmed cases of prion transmission from Cervids to date, there have been several published cases of CJD suspicious for cervid etiology (Angers et al., 2009). The concern of prion transmission from cervids was raised as early as 2001 in a case series that highlighted three separate occurrences of unusually young patients diagnosed with vCJD in the United States (Belay et al., 2001). Two of the patients were frequent hunters and one of the patients was a daughter of a hunter. All three patients had regularly consumed deer and elk meat, which prompted the suspicion for cervid transmission in the absence of any alternative etiology (Belay et al., 2001). Nonetheless, a causal link between the patients’ CJD and their histories of cervid exposure could not be definitively concluded (Belay et al., 2001). A comprehensive review by (Waddell et al., 2017) highlighted two additional case series collectively highlighting 5 patients with CJD who had similar exposures to Cervids without any alternative source explaining their disease.

Prions in deer and elk with CWD are primarily derived directly from antler velvet, which is a highly innervated and vascularized tissue layer that sheds cyclically upon antler ossification (Angers et al., 2009). However, CWD-infected cervids are also capable of shedding prions into the environment through feces, urine and saliva (Hannaoui et al., 2017). In all species, prion disease is caused by the conversion of cellular prion protein (PrPC ) to a pathological “scrapie” prion protein (PrPSC) which rapidly propagates and accumulates in tissue, primarily in the CNS (Iwasaki, 2017; Moreno and Telling, 2018). In humans, this conversion is believed to be from a sporadic or acquired mutation of the PrPC gene PRNP located on the short arm of chromosome 20, which codes for 253 amino acids prior to post-translational modification (Iwasaki, 2017). Multiple mutations of the PRNP gene have been identified that can both cause CJD or increase a host’s susceptibility to prion propagation (Iwasaki, 2017; Nemani et al., 2020). Thus, there is growing concern that stronger CWD variants may exist or arise in cervids breaking ostensible species barriers that may exist (Hannaoui et al., 2017; Nemani et al., 2020; Moreno and Telling, 2018).

Several in vivo animal models have demonstrated plausible cervid to human prion transmission, which is most effectively summarized by (Moreno and Telling, 2017; Angers et al., 2009). Most notably, a 2015 study successfully transmitted CWD prions from elk and mule deer via intracerebral injection into transgenic mice expressing human PrP (Kurt and Sigurdson, 2016). Three published studies- the most recent of which in 2014- have demonstrated successful CWD prion transmission to non-human primates (Waddell et al., 2018). The time from prion exposure to symptom onset varied significantly between studies from 70 days to several months (Waddell et al., 2018). Moreover, (Waddell et al., 2018) also highlighted three successful in vitro studies published demonstrating a strong conversion efficiency of CWD prions on human PrP, the most recent of which published in 2015.

Case Report

A 61 year-old right-handed Caucasian male presented with a one-month history of progressive confusion and gait instability ultimately leading to a 13 day admission. He was found to have moderate cognitive impairment, apathetic affect, left-sided alien hand phenomenon and apraxia, positive Romberg and a dystonic gait pattern with short stride length. The patient was unable to walk without assistance, which prompted an initial suspicion of CBS with unusually rapid progression. The patient also had an extensive history of exotic supplement use going back several years, but notably began taking deer antler velvet and bovine colostrum supplements roughly two months prior. He had no recent travel history, potential occupational exposure to CJD, or family history of prion or neurologic disease. His medical history included hyperlipidemia, distant tobacco use and an incident of diverticulitis several years ago, all of which was considered noncontributory. The patient worked in an office setting, had never been hunting and did not eat deer or elk meet.

Initial Head CT and MRI imaging were unremarkable. A lumbar puncture was performed to evaluate meningitis, autoimmune markers and later 14-3-3 and Rt-QuIC when CJD was suspected (Table 1). A 3-day trial of 1g of Methylprednisolone failed to yield symptomatic improvement and the patient’s presentation continued to progress. Repeat Brain MRI w/ and w/o contrast on day #4 of admission demonstrated asymmetric hyperintense cortical ribboning on diffusion-weighted imaging in the right paramedian frontal and parietal cortex as well as a hyperintensity in the right caudate nucleus (Fig. 1). A subsequent EEG demonstrated periodic triphasic waves typical for CJD patients, which was only transiently attenuated by a trial of benzodiazepines without corresponding symptomatic improvement (Fig. 2) (Iwasaki, 2017; Steinhoff et al., 1996). A third MRI obtained on day #6 of admission showed findings similar to the previous MRI with slightly more hyperintense signal.

The patient continued to demonstrate a progressive decline in neurological status throughout his admission. A Montreal Cognitive Assessment (MoCA) on admission day #5 yielded a score of 17/30 demonstrating significant cognitive impairment (Fig. 3). After failing to reach a definitive diagnosis despite an extensive workup, the patient and his family agreed to discharge to hospice with a likely CJD etiology. Following discharge to hospice, CSF results for protein 14-3-3 and RT-QuIC came back positive, which confirms CJD with 100% specificity (Iwasaki, 2017). A further post-mortem workup is tentative to date and it remains unclear whether this patient’s CJD was sporadic or acquired from exposure to prion-infected cervids or otherwise.

An extensive data query using the lot number and manufacturer information obtained from the patient’s supplements revealed no recalls or reported adverse events of any form from either supplement. Investigation into these supplements showed that the deer antler velvet supplement was curated from a farm in Idaho. There are several known areas significantly impacted by CWD near the Idaho border, which is diligently monitored by state and federal agencies (USGS National Wildlife Health Center, 2020).

Discussion

The bovine-derived CJD outbreak in the United Kingdom of the late 1980s was met with a robust public health and regulatory response and only rare, isolated bovine-derived cases of CJD have been reported in the last 20 years (Iwasaki, 2017). Thus, we believe that it is highly unlikely our patient acquired CJD from the bovine colostrum supplement. Prions specifically from deer antler velvet have been shown to be capable of human transmission through transgenic mouse models, in one case as early as 70 days from inoculation (Moreno and Telling, 2017; Angers et al., 2009). This timeline would support the theory that this patient acquired CJD from the deer antler velvet supplement, as his exposure began at least 60 days prior to the onset of symptoms. It is worth noting that this is the first known case of CJD in which a patient directly consumed deer antler velvet, which is believed to be the primary location of prion propagation in Cervids (Moreno and Telling, 2017; Nemani et al., 2020). This is particularly notable because all other published cases describe cervid exposure from animal meat consumption or hunting.

It is also notable that the patient’s clinical presentation was uniquely similar to Corticobasal Degeneration (CBD) with rapid progression for several reasons. Although myoclonus can be appreciated in both diseases, CJD is known to have a significantly more rapid progression and the diseases are largely distinguishable. However, recently published literature has demonstrated that CBD and CJD share many histological similarities (Iwasaki, 2017). Moreover, it is conceivable that a potential case of CJD acquired from Cervids could yield a unique clinical presentation like that observed in bovine TSE (Moreno and Telling, 2017).

Conclusion

We believe that our patient’s case of CJD is highly suspicious for cervid etiology given the circumstances of the case as well as the strong evidence of plausibility reported in published literature. This is the first known case of CJD in a patient who had consumed deer antler velvet. Despite the confirmed diagnosis of CJD, a causal relationship between the patient’s disease and his consumption of deer antler velvet cannot be definitively concluded.

Supplemental data including molecular tissue sample analysis and autopsy findings could yield further supporting evidence. Given this patient’s clinical resemblance to CBD and the known histological similarities of CBD with CJD, clinicians should consider both diseases in the differential diagnosis of patients with a similarly esoteric presentation. Regardless of the origin of this patient’s disease, it is clear that the potential for prion transmission from cervids to humans should be further investigated by the academic community with considerable urgency.

https://thescipub.com/pdf/ajidsp.2021.43.48.pdf

''We believe that our patient’s case of CJD is highly suspicious for cervid etiology given the circumstances of the case as well as the strong evidence of plausibility reported in published literature. This is the first known case of CJD in a patient who had consumed deer antler velvet. Despite the confirmed diagnosis of CJD, a causal relationship between the patient’s disease and his consumption of deer antler velvet cannot be definitively concluded.''

i believe that there is more than enough evidence to think of this cases as highly possible cwd zoonosis from deer antler velvet... imo...terry

SUNDAY, JULY 24, 2016

Chronic Wasting Disease Prions in Elk Antler Velvet and Marketing of this Product in Nutritional Supplements for Humans?

Research Project: TRANSMISSIBLE SPONGIFORM ENCEPHALOPATHIES: THE ROLE OF GENETICS, STRAIN VARIATION, AND ENVIRONMENTAL CONTAMINATION IN DISEASE CONTROL

Title: Chronic Wasting Disease Prions in Elk Antler Velvet

Authors

item Angers, R - UNIVERSITY OF KENTUCKY item Napier, D - UNIVERSITY OF KENTUCKY item Seward, T - UNIVERSITY OF KENTUCKY item Green, M - UNIVERSITY OF KENTUCKY item Spraker, T - COLORADO STATE UNIVERSITY item O'Rourke, Katherine item Balachandran, A - CANADIAN FOOD INSPCTN AG item Telling, G - UNIVERSITY OF KENTUCKY

Submitted to: Emerging Infectious Diseases Publication Type: Peer Reviewed Journal Publication Acceptance Date: February 1, 2009

Publication Date: May 1, 2009 Repository URL: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2687044/pdf/08-1458_finalR.pdf

Citation: Angers RC, Seward TS, Napier D, Green M, Hoover E, Spraker T, et al. 2009. Chronic wasting disease prions in elk antler velvet. Emerg Infect Dis 15(5):696-703.

Interpretive Summary: Chronic wasting disease (CWD) is a fatal neurologic disease of deer and elk in the United States and Canada. The disease is associated with accumulations of infectious proteins in the brain, nervous system, blood, and a limited number of other tissues. In this study, the investigators examined elk antler velvet, the covering that grows on elk antlers every year. Antler velvet is rich in blood and nervous supply and may represent a source of infectious material as the velvet is shed every year. Antler velvet and brain tissue from four infected elk was examined by immunohistochemistry and biochemical methods, with no evidence of the abnormal prion protein in antler velvet. The same preparations were tested in genetically engineered mice susceptible to CWD. Mice in both inoculated groups developed prion disease. This finding demonstrates that antler velvet from CWD infected elk contain infectious material and may represent a risk material to other elk. Technical Abstract: Chronic wasting disease (CWD) is a transmissible spongiform encephalopathy or prion disease of captive and free ranging white tailed deer, mule deer, Rocky Mountain elk and moose in the some parts of the United States and Canada. The presence of the disease has sharply curtailed movement of captive animals and reduced the domestic or international market for some cervid by-products. The disease appears to be transmitted by Rocky Mountain elk relatively late in the disease course, but the sources of the infectious material remain undefined. Brain and lymphoid tissue contain the highest levels of the abnormal prion protein, the marker for disease, and transmission in white tailed deer can be accomplished by blood transfusion from experimentally infected deer to naive deer. In this study, the investigators examined the transmission potential of antler velvet, a highly vascularized and innervated epidermal tissue covering the growing antler. Antler velvet is shed each year and is widely used as a nutritional supplement. Genetically engineered mice susceptible to CWD were inoculated with homogenates of paired brain and antler velvet from 4 elk with CWD. Mice in both groups developed a transmissible spongiform encephalopathy. These findings demonstrate prion infectivity accumulates in antler velvet and may have impact on marketing of this product.

Last Modified: 7/24/2016

http://www.ars.usda.gov/research/publications/publications.htm?SEQ_NO_115=227882

Chronic Wasting Disease Prions in Elk Antler Velvet

Rachel C. Angers,1 Tanya S. Seward, Dana Napier, Michael Green, Edward Hoover, Terry Spraker, Katherine O’Rourke, Aru Balachandran, and Glenn C. Telling

Chronic wasting disease (CWD) is a contagious, fatal prion disease of deer and elk that continues to emerge in new locations. To explore the means by which prions are transmitted with high efficiency among cervids, we examined prion infectivity in the apical skin layer covering the growing antler (antler velvet) by using CWD-susceptible transgenic mice and protein misfolding cyclic amplification. Our finding of prions in antler velvet of CWD-affected elk suggests that this tissue may play a role in disease transmission among cervids. Humans who consume antler velvet as a nutritional supplement are at risk for exposure to prions. The fact that CWD prion incubation times in transgenic mice expressing elk prion protein are consistently more rapid raises the possibility that residue 226, the sole primary structural difference between deer and elk prion protein, may be a major determinant of CWD pathogenesis.

snip...

Implications for Horizontal CWD Transmission and Human Exposure

Our studies indicate that antler velvet represents a previously unrecognized source of CWD prions in the environment. Whereas oral transmission of rodent-adapted scrapie prions is known to be ≈5 orders of magnitude less efficient than transmission by intracerebral inoculation (14,15), the relative efficiency of oral CWD prion transmission is unknown. Multiple exposures to low levels of CWD prions in the environment (16,17), as well as increased infectivity when prions are bound to soil minerals (18), are factors that may influence transmission.

The appearance of variant Creutzfeldt-Jakob disease in humans exposed to bovine spongiform encephalopathy (BSE) (19,20) and the demonstration of CWD prions in muscle (3) placed the human species barrier to CWD prions at the forefront of public health concerns. Our studies indicate that antler velvet represents an additional source for human exposure to CWD prions. Widely used in traditional Asian medicine to treat a variety of ailments including impotence, arthritis, and high blood pressure, antler velvet can be readily purchased in caplet form and its usage has increased worldwide.

Fortunately, to date there is no epidemiologic evidence that rates of CJD in the CWD-endemic region (Colorado, USA) have increased (21,22). Also reassuring is the inefficient in vitro conversion of human PrP to protease-resistant PrP by CWD (23). Two studies have shown that CWD prions failed to induce disease in Tg mice expressing human PrP (24,25). However, the failure of BSE to be transmitted to Tg mice expressing human prion protein (HuPrP) was cited as early evidence of a BSE transmission barrier in humans (26); subsequent studies demonstrated a strong effect of the codon 129 polymorphism on transmissibility of BSE prions (27). To date, only mice expressing HuPrP with methionine at 129 have been challenged with CWD. In support of the argument that humans might be susceptible to CWD, intracerebral inoculation of squirrel monkeys produced disease after >30 months (28). Prion strain properties are also critical when considering the potential for interspecies transmission. The existence of multiple CWD strains has been suggested by several studies (4,25,29,30), but strain isolation and host range characterization have not been reported. Finally, it is worth considering that if CWD were to cross the species barrier into humans, this transmission source might not be recognized if the disease profile overlapped with one of the forms of sporadic CJD reported in North America.

snip...end

http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2687044/pdf/08-1458_finalR.pdf

Volume 15, Number 5—May 2009

Research

Chronic Wasting Disease Prions in Elk Antler Velvet

http://wwwnc.cdc.gov/eid/article/15/5/08-1458_article.htm

http://chronic-wasting-disease.blogspot.com/2009/03/chronic-wasting-disease-prions-in-elk.html

http://chronic-wasting-disease.blogspot.com/2012/02/new-supplement-from-deer-antler-velvet.html

http://bovineprp.blogspot.com/2015/08/federal-judge-enters-permanent.html

ABOUT that deer antler spray and CWD TSE PRION...

I have been screaming this since my neighbors mom died from cjd, and she had been taking a supplement that contained bovine brain, bovine eyeball, and other SRMs specified risk materials, the most high risk for mad cow disease.

just saying...

I made a submission to the BSE Inquiry long ago during the BSE Inquiry days, and they seemed pretty interested.

Sender: "Patricia Cantos"

To: "Terry S Singeltary Sr. (E-mail)"

Subject: Your submission to the Inquiry

Date: Fri, 3 Jul 1998 10:10:05 +0100

3 July 1998

Mr Terry S Singeltary Sr.

E-Mail: Flounder at wt.net

Ref: E2979

Dear Mr Singeltary,

Thank you for your E-mail message of the 30th of June 1998 providing the Inquiry with your further comments.

Thank you for offering to provide the Inquiry with any test results on the nutritional supplements your mother was taking before she died.

As requested I am sending you our general Information Pack and a copy of the Chairman's letter. Please contact me if your system cannot read the attachments.

Regarding your question, the Inquiry is looking into many aspects of the scientific evidence on BSE and nvCJD. I would refer you to the transcripts of evidence we have already heard which are found on our internet site at ;

http://www.bse.org.uk.

Could you please provide the Inquiry with a copy of the press article you refer to in your e-mail? If not an approximate date for the article so that we can locate it?

In the meantime, thank you for you comments. Please do not hesitate to contact me on...

snip...end...tss

everyone I tell this too gets it screwed up...MY MOTHER WAS NOT TAKING THOSE SUPPLEMENTS IPLEX (that I ever knew of). this was my neighbors mother that died exactly one year _previously_ and to the day of sporadic CJD that was diagnosed as Alzheimer’s at first. my mother died exactly a year later from the Heidenhain Variant of Creutzfeldt Jakob Disease hvCJD, and exceedingly rare strains of the ever growing sporadic CJD’s. _both_ cases confirmed. ...kind regards, terry

TSEs i.e. mad cow disease's BSE/BASE and NUTRITIONAL SUPPLEMENTS

IPLEX, mad by standard process;

vacuum dried bovine BRAIN, bone meal, bovine EYE, veal Bone, bovine liver powder, bovine adrenal, vacuum dried bovine kidney, and vacuum dried porcine stomach.

also;

what about potential mad cow candy bars ?

see their potential mad cow candy bar list too...

THESE are just a few of MANY of just this ONE COMPANY...TSS

DEPARTMENT OF HEALTH AND HUMAN SERVICES

FOOD AND DRUG ADMINISTRATION CENTER FOR BIOLOGICS EVALUATION AND RESEARCH

TRANSMISSIBLE SPONGIFORM ENCEPHALOPATHIES ADVISORY COMMITTEE

Friday, January 19, 2001 snip...

17 But I think that we could exhibit some quite

18 reasonable concern about blood donors who are taking dietary

19 supplements that contain a certain amount of unspecified-

20 origin brain, brain-related, brain and pituitary material.

21 If they have done this for more than a sniff or something

22 like that, then, perhaps, they should be deferred as blood

23 donors.

24 That is probably worse than spending six months in

25 the U.K.

1/19/01

3681t2.rtf(845) page 501

http://www.fda.gov/ohrms/dockets/ac/cber01.htm

http://www.fda.gov/ohrms/dockets/dailys/03/Mar03/031403/96N-0417-EC-2.htm

http://www.fda.gov/ohrms/dockets/ac/01/slides/3681s2_07.pdf

see full text ;

http://chronic-wasting-disease.blogspot.com/2009/03/chronic-wasting-disease-prions-in-elk.html

TSEs i.e. mad cow disease's BSE/BASE and NUTRITIONAL SUPPLEMENTS

IPLEX, mad by standard process;

vacuum dried bovine BRAIN, bone meal, bovine EYE, veal Bone, bovine liver powder, bovine adrenal, vacuum dried bovine kidney, and vacuum dried porcine stomach.

also;

what about potential mad cow candy bars ?

see their potential mad cow candy bar list too...

THESE are just a few of MANY of just this ONE COMPANY...TSS

DEPARTMENT OF HEALTH AND HUMAN SERVICES

FOOD AND DRUG ADMINISTRATION CENTER FOR BIOLOGICS EVALUATION AND RESEARCH

TRANSMISSIBLE SPONGIFORM ENCEPHALOPATHIES ADVISORY COMMITTEE

Friday, January 19, 2001 snip...

17 But I think that we could exhibit some quite

18 reasonable concern about blood donors who are taking dietary

19 supplements that contain a certain amount of unspecified-

20 origin brain, brain-related, brain and pituitary material.

21 If they have done this for more than a sniff or something

22 like that, then, perhaps, they should be deferred as blood

23 donors.

24 That is probably worse than spending six months in

25 the U.K.

1/19/01

3681t2.rtf(845) page 501

http://www.fda.gov/ohrms/dockets/ac/cber01.htm

Docket Management Docket: 96N-0417 - Current Good Manufacturing Practice in Manufacturing, Packing, or Holding Dietary Ingredients a Comment Number: EC -2 Accepted - Volume 7

http://www.fda.gov/ohrms/dockets/dailys/03/Mar03/031403/96N-0417-EC-2.htm

http://www.fda.gov/ohrms/dockets/ac/01/slides/3681s2_07.pdf

see full text ;

http://chronic-wasting-disease.blogspot.com/2009/03/chronic-wasting-disease-prions-in-elk.html

2003 - 2004 Product Catalog

Standard Process Inc.

NATURAL COCOA STANDARDBAR (mad cow candy bar) (i will just list animal organs) bovine adrenal, bovine liver, bovine spleen, ovine spleen, bovine kidney...

NATURAL PEANUT BUTTER STANDARDBAR

bovine adrenal, bovine liver, bovine spleen, ovine spleen, bovine kidney...

USF (MAD COW) OINTMENT (RUB A DUB DUB, KURU ETC) ;

bovine orhic glandular extract

UTROPHIN PMG

bovine uterus PMG

VASCULIN

bovine heart PMG extract, veal bone PMG extract, bovine liever, porcine duodenum, bovine adrenal Cytosol extract, bovine spleen, ovine spleen

IPLEX (neighbors mom died from CJD while taking these pills for years)

bovine eye PMG extract, veal bone PMG, bovine liver, porcine stomach, bovine adrenal, bovine kidney, bovine adrenal Cytosol extract, BOVINE BRAIN, bovine bone, veal bone meal

MYO-PLUS

bovine heart PMG, bovine liver, porcine stomach, bovine orchic extract, bovine spleen, ovine spleen, bovine adrenal Cytosol extract, BOVINE BRAIN

NEUROPLEX

bovine orchic Cytosol extract, bovine spleen, BOVINE BRAIN PMG EXTRACT, BOVINE ANTERIOR PITUITARY, bovine liver, BOVINE PITUITARY PMG EXTRACT, AND MORE BOVINE BRAIN...

NEUROTROPHIN PMG

BOVINE BRAIN PMG

NIACINAMIDE B6 VM

bovine liver, porcine stomach, bovine spleen ovine spleen, BOVINE BRAIN

OCULOTROPHIN PMG BOVINE EYE PMG

ORCHEX

bovine liver, bovine orchic Cytosol extract, porcine stomch, bovine spleen, ovine spleen, BOVINE BRAIN

OSTARPLEX

veal bone PMG extract, veal bone PMG extract, bovine liver, porcine stomach, bovine adrenal, bovine spleen, ovine spleen, BOVINE BRAIN

PARAPLEX

bovine pancreas PMG extract, porcine duodenum, bovine adrenal PMG, BOVINE PITUITARY PMG EXTRACT, bovine thyroid PMG extract

PITUITROPHIN PMG

RUMAPLEX

BOVINE BRAIN, veal bone PMG extract, bovine adrenal, bovine prostate Cytosol extract, veal bone meal, bovine liver PMG extract, bovine spleen, ovine spleen, bovine liver

SENAPLEX

bovine liver PMG extract, bovine adrenal, BOVNE BRAIN, veal bone meal, bovine kidney, bovine orchic extract, bovine spleen, ovine spleen ..........

THESE are just a few of MANY of just this ONE COMPANY.

FOR the following reason, I implore that the FDA take serious action in further protecting the consumer from the TSE agent via nutritional supplements.

Does all that e-mail spam promising sexual vitality actually hide serious risk of contracting MAD COW DISEASE?

Volume 361, Number 9368 03 May 2003

Correspondence

Tighter regulation needed for dietary supplements in USA

Sir--Mary Palmer and colleagues (Jan 11, p 101)1 found that dietary supplements have the potential to cause serious adverse effects. The investigators state that research on the hazards and risks of dietary supplements should be a priority. The safety of individuals who consume these products is important, and organisations such as the US Food and Drug Administration (FDA) need to take initiative by enforcing stricter regulations on supplements. Several commonly used products--for example ginkgo biloba, St John's Wort, and ephedrine--can have serious adverse effects.2 Although the FDA requires multiple studies on the safety and efficacy for pharmaceutical products before placing them on the market, standards are less robust for dietary supplements. In the USA, under the Dietary Supplement Health and Education Act (DSHEA) of 1994, supplements are subject to the same regulatory requirements as food. There are no provisions that require FDA approval for the safety or effectiveness of supplements,3 which leaves consumers and manufacturers essentially responsible for the health effects of these products. The DSHEA of 1994 needs to be revised so that dietary supplements are subject to the same regulations as pharmacological drugs. The FDA needs to review clinical studies on the safety and efficacy of dietary supplements. Organisations such as Public Citizen and the American Medical Association are already taking steps to achieve these changes. However, they face immense opposition from groups such as the National Nutritional Foods Association, the American Herbal Association, and the Council for Responsible Nutrition. To overcome such resistance, consumer organisations, health-care providers, and government agencies need to approach this subject in unison. The public needs to be able to assess the risks and benefits of dietary supplements before consuming them. Health-care providers and the more than 100 million Americans who consume these products4 should encourage the FDA to treat supplements with the stringent regulations it enforces on pharmaceutical products.

Nipa Kinariwala

----------------------------------------------------------

700 Bolinwood Drive, Apartment 12A, Chapel Hill, NC 27514, USA (e-mail nskinari at aol.com) 1 Palmer ME, Haller C, McKinney PE, et al. Adverse events associated with dietary supplements: an observational study. Lancet 2003; 361: 101-06. [Text ] 2 Cupp MJ. Herbal remedies: adverse effects and drug interactions. Am Fam Physician 1999; 59: 1239-45. [PubMed ] 3 Unites States Food and Drug Administration. Overview of dietary supplements. Jan 3, 2001. http://www.cfsan.fda.gov/~dms/ds-oview.html (accessed Feb 20, 2002). 4 Pear R. Feds call for tighter control over nutritional supplements. Organic Consumers Association, April 17, 2001. http://www.organicconsumers.org/Organic/dietsupp.cfm (accessed Feb 20, 2002).

http://www.thelancet.com/journal/vol361/iss9368/full/llan.361.9368.correspondence.25505.1

snip...end tss letter to fda.

=================== 2013 ================

my plight with Metabolife, the GAO et al, and my submission to the BSE inquiry, about mad cow in a pill ;

Wednesday, March 20, 2013

GAO-13-244, Mar 18, 2013 Dietary Supplements FDA May Have Opportunities to Expand Its Use of Reported Health Problems to Oversee Product

From: Terry S. Singeltary Sr.

Sent: Tuesday, March 19, 2013 2:46 PM

To: mailto:gomezj%40gao.gov Cc: mailto:siggerudk%40gao.gov ; mailto:youngc1%40gao.gov ; mailto:oighotline%40gao.gov

http://transmissiblespongiformencephalopathy.blogspot.com/2013/03/gao-13-244-mar-18-2013-dietary.html

Monday, February 01, 2010

Import Alert 17-04 BSE CJD HIGH RISK TISSUES, Nutritional Supplements and Cosmetics Import Alert 17-04

http://creutzfeldt-jakob-disease.blogspot.com/2010/02/import-alert-17-04-bse-cjd-high-risk.html

Thursday, March 19, 2009

Chronic Wasting Disease Prions in Elk Antler Velvet (Nutritional Supplements and CJD)

10.3201/eid1505.081458 Suggested citation for this article: Angers RC, Seward TS, Napier D, Green M, Hoover E, Spraker T, et al. Chronic wasting disease prions in elk antler velvet. Emerg Infect Dis. 2009 May; [Epub ahead of print]

http://chronic-wasting-disease.blogspot.com/2009/03/chronic-wasting-disease-prions-in-elk.html

Docket Management Docket: 96N-0417 - Current Good Manufacturing Practice in Manufacturing, Packing, or Holding Dietary Ingredients a

Comment Number: EC –2

Accepted - Volume 7

http://www.fda.gov/ohrms/dockets/dailys/03/Mar03/031403/96N-0417-EC-2.htm

Docket Management Docket: 96N-0417 - Current Good Manufacturing Practice in Manufacturing, Packing, or Holding Dietary Ingredients a Comment Number: EC -2 Accepted - Volume 7

snip...

what did Paul Brown say about this previously;

i bring your attention to (page 500) Dr. Paul Brown statements;

253 1 DR. BOLTON: I have an additional question about 2 that. What is the assurance that additional locally sourced 3 tracheas are not added into that manufacturing process, thus 4 boosting the yield, if you will, but being returned to the 5 U.S. as being produced from U.S.-sourced raw material? 6 DR. McCURDY: Are there data to indicate how many 7 grams, or whatever, of infected brain are likely to infect 8 an organism, either animal or man, when taken orally? 9 DR. BROWN: If I am not mistaken, and I can be 10 corrected, I think a half a gram is enough in a cow, orally; [FULL TEXT ABOUT 600 PAGES] 3681t2.rtf

http://www.fda.gov/ohrms/dockets/ac/cber01.htm

snip...

http://www.fda.gov/OHRMS/DOCKETS/DOCKETS/96n0417/96N-0417-EC-2.htm

Unregulated "foods" such as 'nutritional supplements' containing various extracts from ruminants, whether imported or derived from 3 US cattle/sheep/cervids ("antler velvet" extracts!) should be forbidden or at least very seriously regulated.

(neighbors Mom, whom also died from CJD, had been taking bovine based supplement, which contained brain, eye, and many other bovine/ovine tissues for years, 'IPLEX').

http://www.fda.gov/OHRMS/DOCKETS/AC/01/slides/3681s2_09.pdf

my plight with metabolife and there 'bovine complex' about risk factors of TSE in there product ;

Terry S. Singeltary Sr. wrote:

######## Bovine Spongiform Encephalopathy > > #########

1. Dietary Supplements: Review of Health-Related Call Records for

Users of Metabolife 356. GAO-03-494, March 31.

http://www.gao.gov/cgi-bin/getrpt?GAO-03-494

http://www.gao.gov/highlights/d03494high.pdf

-------- Original Message --------

Subject: METABOLIFE AND TSEs GAO-03-494 ''URGENT DATA''

Date: Thu, 01 May 2003 11:23:01 –0500

From: "Terry S. Singeltary Sr."

To: NelliganJ at gao.gov

The General Accounting Office (GAO) today released the following reports and testimonies: ‘'

REPORTS;

1. Dietary Supplements: Review of Health-Related Call Records for

Users of Metabolife 356. GAO-03-494, March 31.

http://www.gao.gov/cgi-bin/getrpt?GAO-03-494

http://www.gao.gov/highlights/d03494high.pdf

GREETINGS GAO:

i was suprised that i did not see any listing of bovine tissue in > metabolife on it's label. have they ceased using these desiccated tissues???

i see that the lable on this product METABOLIFE 356, does not state that it has any tissues of desiccated bovine organs? i no > the product use to, so i am curious if they have ceased the use of the tissues of cattle they _use_ to use (see below)???

METABOLIFE 356

BOVINE COMPLEX/GLANDULAR SYSTEM

OVARIES, PROSTATE, SCROTUM AND ADRENAL

USDA SOURCE CATTLE

i tried warning them years ago of this potential threat of CJD/TSEs;

From: Randy Smith

To: "'flounder at wt.net'"

Subject: Metabolife

Date: Mon, 7 Dec 1998 14:21:35 –0800

Dear Sir,

We are looking at reformulation. I agree that slow virus diseases present a problem in some areas of the world.

Our product uses healthy USDA inspected cattle for the glandular extract.

If you have any links to more information on this subject I would like to examine them.

Thank you for your interest and concern,

Dr. Smith

============

From: Randy Smith

To: "'flounder at wt.net'"

Subject: RE: [Fwd: Your submission to the Inquiry]

Date: Wed, 9 Dec 1998 10:37:07 –0800

Terry,

Thank you for your note and the information links you forwarded to me.

I am new to Metabolife International, however hopefully as my role here enlarges I well have a greater impact on formulation and product development.

Metabolife International does believe in placing safety first. And I am going to do my best to see that we continue to do so.

Sincerely,

Dr. Smith

============

-----Original Message-----

From: Terry S. Singeltary Sr. [mailto:flounder at wt.net]

Sent: Wednesday, December 09, 1998 5:49 PM

To: rsmith at metabolife.com

Subject: [Fwd: Your submission to the Inquiry]

Dr. Smith, I am truly impressed with you honesty, THANKS.....I am not just spouting off about the potential dangers, here. THEY ARE REAL.....I have forwarded an e-mail from the BSE Inquiry, in which I made a statement about them........You might want to go to the site and read through it........IT WILL TAKE A WHILE........ THINGS ARE HAPPENING HERE SIR, THAT YOU ARE NOT AWARE OF, AND AS MOST PEOPLE ARE NOT...............I JUST HOPE, THAT THE REFORMULATION YOU SPEAK OF, IS IN FACT GOING TO TAKE PLACE.

The Department of Health, here in the U.S., is also worried about the potential dangers involved hear............Terry/MADSON

==================================================

From: Randy Smith

To: "'flounder at wt.net'"

Subject: RE: [Fwd: MEDICINES "GREATER BSE RISK THAN BEEF"!!!!]

Date: Fri, 18 Dec 1998 09:55:17 –0800

Return-Receipt-To: Randy Smith

Thanks very much for the info. I appreciate all these articles I can get. It does sound very familiar - just follow the green ($) trail.

-----Original Message-----

From: Terry S. Singeltary Sr. [mailto:flounder at wt.net]

Sent: Friday, December 18, 1998 5:15 PM

To: rsmith at metabolife.com

Subject: [Fwd: MEDICINES "GREATER BSE RISK THAN BEEF"!!!!]

Randy, thought you might be interested in > > this...............MADSON!!!!!1

snip...

===============================

-------- Original Message --------

Subject: re: METABOLIFE AND TSEs GAO-03-494 ''URGENT DATA''

Date: Thu, 01 May 2003 16:04:35 –0400

From: "Marcia G Crosse"

To:

CC: "Charles W Davenport" , "Carolyn Feis Korman" > > , "Martin Gahart" >

Mr. Singletary,

We were informed by representatives of Metabolife, Inc. that Metabolife 356 was reformulated to remove bovine complex as an ingredient in the product, approximately September 2001. We did not independently verify the contents of the product.

Sincerely,

Marcia Crosse

Acting Director

Health CarePublic Health and Science Issues

U.S. General Accounting Office

441 G Street, N.W.

Washington, D.C. 20548

===================

-------- Original Message --------

Subject: Re: METABOLIFE AND TSEs GAO-03-494 ''URGENT DATA''

Date: Thu, 01 May 2003 15:48:52 –0500

From: "Terry S. Singeltary Sr."

To: Marcia G Crosse

CC: Charles W Davenport , Carolyn Feis Korman > > , Martin Gahart > References: > >

THANK YOU!

MIRACLES DO HAPPEN! ;-)

now all we need to do is;

snip......

one small step for man, one giant leap for mankind ;-)

however;

''We did not independently verify the contents of the product''

???

TSS

####### http://mailhost.rz.uni-karlsruhe.de/warc/bse-l.html ########

http://bseinquiry.blogspot.com/

http://www.fda.gov/ohrms/dockets/dailys/03/Mar03/031403/96N-0417-EC-2.htm

Could you get mad cow from a pill ? Some doctors say a class of pills that promise smarts, energy, and sexual vitality may cause mad-cow disease.

The government isn't worried. Should you be?

June 1, 2001

Health Magazine

by Susan Freinkel

http://www.organicconsumers.org/madcow/pill6101.cfm

The German Magazine Der Spiegel came out to the house here and interviewed me in 2001 (I think), about that token purina mad cow feed mill blunder, and they were very concerned about these type supplements that carried the SRMs that could very well carry the TSE prion agent. ...please see ;

GERMAN DER SPIEGEL MAGAZINEDie

BSE-Angst erreicht Amerika: Trotz strikter Auflagen gelangte in Texas verbotenes Tiermehl ins Rinderfutter - die Kontrollen der Aufsichtsbehörden sind lax.

http://www.spiegel.de/spiegel/0,1518,119306,00.html

DER SPIEGEL (9/2001) - 24.02.2001 (9397 Zeichen) USA: Loch in der Mauer Die BSE-Angst erreicht Amerika: Trotz strikter Auflagen gelangte in Texas verbotenes Tiermehl ins Rinderfutter - die Kontrollen der Aufsichtsbehördensind lax.Link auf diesen Artikel im Archiv:

http://service.spiegel.de/digas/find?DID=18578755

"Löcher wie in einem Schweizer Käse" hat auch Terry Singeltary im Regelwerk der FDA ausgemacht. Der Texaner kam auf einem tragischen Umweg zu dem Thema: Nachdem seine Mutter 1997 binnen weniger Wochen an der Creutzfeldt-Jakob-Krankheit gestorben war, versuchte er, die Ursachen der Infektion aufzuspüren. Er klagte auf die Herausgabe von Regierungsdokumenten und arbeitete sich durch Fachliteratur; heute ist er überzeugt, dass seine Mutter durch die stetige Einnahme von angeblich kräftigenden Mitteln erkrankte, in denen - völlig legal - Anteile aus Rinderprodukten enthalten sind.

Von der Fachwelt wurde Singeltary lange als versponnener Außenseiter belächelt. Doch mittlerweile sorgen sich auch Experten, dass ausgerechnet diese verschreibungsfreien Wundercocktails zur Stärkung von Intelligenz, Immunsystem oder Libido von den Importbeschränkungen ausgenommen sind. Dabei enthalten die Pillen und Ampullen, die in Supermärkten verkauft werden, exotische Mixturen aus Rinderaugen; dazu Extrakte von Hypophyse oder Kälberföten, Prostata, Lymphknoten und gefriergetrocknetem Schweinemagen. In die USA hereingelassen werden auch Blut, Fett, Gelatine und Samen. Diese Stoffe tauchen noch immer in US-Produkten auf, inklusive Medizin und Kosmetika. Selbst in Impfstoffen waren möglicherweise gefährliche Rinderprodukte enthalten. Zwar fordert die FDA schon seit acht Jahren die US-Pharmaindustrie auf, keine Stoffe aus Ländern zu benutzen, in denen die Gefahr einer BSE-Infizierung besteht. Aber erst kürzlich verpflichteten sich fünf Unternehmen, darunter Branchenführer wie GlaxoSmithKline, Aventis und American Home Products, ihre Seren nur noch aus unverdächtigem Material herzustellen.

"Its as full of holes as Swiss Cheese" says Terry Singeltary of the FDA regulations. ...

http://www.spiegel.de/spiegel/print/d-18578755.html

http://wissen.spiegel.de/wissen/image/show.html?did=18578755&aref=image024/E0108/SCSP200100901440145.pdf&thumb=false

http://service.spiegel.de/digas/servlet/find/DID=18578755

Sunday, November 10, 2013

LARGE CJD TSE PRION POTENTIAL CASE STUDY AMONG HUMANS WHO TAKE DEER ANTLER VELVET WILL BE ONGOING FOR YEARS IF NOT DECADES, but who's cares $

http://creutzfeldtejakob-disease.blogspot.com/2013/11/large-cjd-tse-prion-potential-case.html

http://chronic-wasting-disease.blogspot.com/2016/07/chronic-wasting-disease-prions-in-elk.html

https://chronic-wasting-disease.blogspot.com/2012/02/new-supplement-from-deer-antler-velvet.html

https://cjdquestionnaire.blogspot.com/2015/10/cjd-foundation-creutzfeldt-jakob.html

Terry S. Singeltary Sr., Bacliff, Texas USA, Galveston Bay, flounder9@verizon.net on the bottom!