-----Original Message-----
From: CFIA Webmaster
Sent: Monday, April 02, 2012 5:37 PM
To: CFIA-AD_ACIA-MA@WWW.AGR.GC.CA
Subject: 20120402 - Breach of quarantine/Violation de la mise en quarantaine
Le texte français suit le texte anglais.
Breach of Quarantine During Animal Disease Investigation
The Canadian Food Inspection Agency (CFIA) is working with provincial police to locate sheep that have been removed from a farm currently under a quarantine order. The sheep were quarantined as part of an ongoing scrapie investigation at a farm in Eastern Ontario.
http://www.inspection.gc.ca/eng/1333403826731/1333404034371
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Violation de la mise en quarantaine au cours d’une enquête sur la maladie animale
L’Agence canadienne d’inspection des aliments (ACIA) collabore avec la police provinciale afin de retracer des moutons qui ont été retirés d’une exploitation agricole en violation de l’ordonnance da mise en quarantaine. Les moutons avaient été mis en quarantaine dans le cadre d’une enquête en cours sur la tremblante du mouton dans une ferme de l’Est de l’Ontario.
http://www.inspection.gc.ca/fra/1333403826731/1333404034371
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CFIA bowing to Americans: lawyer
Missing sheep
By Mark Hoult QMI Agency
Posted 5 hours ago
If a judgement call must be made over the slaughter of 41 sheep, civil libertarian lawyer Karen Selick argues the call should be in favour of private property rights, in favour of the farmer and in favour of the genetic diversity of the sheep.
Instead, the CFIA is bowing to international regulations, in particular those of the United States, said Selick, said Selick, Canadian Constitution Foundation litigation director, who practises law in Belleville. “They want to do what the U.S. is doing and telling them to do, because the U.S. does not have its borders open to Canadian sheep. So it's about U.S. protectionism.”
But Canadian chief veterinary officer Brian Evans said Jones's sheep are suspected of having scrapie. “The Canadian Food Inspection Agency is committed to protecting livestock health, and takes the management of animal diseases very seriously,” he said. “While we recognize that disease control activities can be difficult on producers, the eradication of animal diseases, such as scrapie, is critical to ensuring the long-term sustainability of the sheep industry.”
Small Ruminant Veterinarians of Ontario representative Dr. Paula Menzies said the organization supports the eradication of scrapie. “Although we sympathize with owners of affected flocks, Canada must deal effectively with this disease.”
However, the CFIA offered no comments on why it believes Jones' farm is infected with scrapie or why it decided to slaughter the 41 animals instead of negotiating an alternative solution. In an e-mailed response to questions, CFIA media relations officer Lisa Gauthier would say only that when scrapie is suspected or confirmed on a farm, it is placed under strict quarantine “and all infected and at-risk animals are ordered humanely destroyed and disposed of.” The measures are based on internationally accepted science, she said.
“This particular farm has been under quarantine since late January 2010, when scrapie was first suspected there,” Gauthier added. “Quarantines are necessary to control the spread of the disease.”
Gauthier said that because scrapie is a disease listed by the World Organization for Animal Health “Canada has international and trade obligations to respond to suspected cases.”
The CFIA initiated its National Scrapie Eradication Program in 2005, she said. The program “is supported by the small ruminant industry” and consists of an internationally recognized and science-based approach that involves a national surveillance program to identify as many infection sites as possible, along with the implementation of actions to control scrapie on farms where it has been identified.
http://www.intelligencer.ca/ArticleDisplay.aspx?e=3525236
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Scrapie-quarantined sheep vanish from Ont. farm
CFIA was coming to destroy rare sheep, farmer's lawyer says
Apr 3, 2012 6:36 AM - 7 comments
TEXT SIZE
By: Staff
Livestock
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Ontario Provincial Police and the Canadian Food Inspection Agency are looking for a flock of sheep that has disappeared from a southeastern Ontario farm under quarantine for scrapie.
The CFIA said in a release Monday that the animals in question are "suspected of having scrapie," a federally reportable nerve disease related to BSE in cattle and Creutzfeldt-Jakob disease in people.
The sheep were found to be missing the same day 41 of the "apparently healthy" animals, including 20 pregnant ewes, were scheduled to be destroyed by CFIA order. That's according to an earlier release from Karen Selick, a lawyer for the Calgary-based Canadian Constitution Foundation, representing producer Montana Jones.
Local media show Jones' farm at Trent Hills, about 50 km east of Peterborough, has attracted a number of protesters urging a reprieve for the sheep.
According to a news report Monday from Peterborough TV station CHEX, an unknown party took a number of the sheep from Jones' barn sometime between Sunday evening and Monday morning and left a note saying the animals were in "protective custody."
All of the condemned animals tested by CFIA previously tested negative for scrapie in live biopsies, and none of the animals had shown clinical symptoms of the disease in the 12 years Jones has raised sheep, the foundation previously alleged.
A single sheep Jones sold to an Alberta farm in 2007 was later found to have scrapie, the foundation said, alleging scientists can't accurately determine when or where it acquired the illness.
Jones' farm, the foundation said, has "nevertheless been under quarantine" since January 2009, causing "great financial hardship."
Jones, in the foundation's release last week, described her sheep as Shropshires, an "endangered breed," noting "they're due to have lambs soon so I'm expecting 30 to 40 new babies. If CFIA kills my pregnant mothers, there will be only 107 or so females left in Canada."
Jones said CFIA staff have rejected her proposed "alternative risk-control measures," such as taking 30 sheep for destruction and testing while allowing her to keep back 11 of "the most significant rare breeding stock."
"They have also been refusing to allow a third party tissue test. They plan to take away the only evidence I might have to disprove their results if they claim there is a positive," Jones alleged in the foundation's release. "I have seen the CFIA make numerous errors and am very concerned that their results could be inaccurate."
"The government therefore has a duty not to act arbitrarily or disproportionately, but in our view it is doing both," Selick said in her release.
"We sympathize"
Shropshire breeding animals are classified with livestock conservation group Rare Breeds Canada (RBC) and the Rare Breeds Trust of Australia as being in "critical" low numbers. The U.K.-based Rare Breeds Survival Trust designates Shropshires as a "minority."
Jones is not listed with RBC as a Shropshire breeder. However, RBC livestock chairman Elwood Quinn, a Quebec producer, recently said on the organization's website he hopes "common sense will prevail" in the Jones case.
"While we recognize that disease control activities can be difficult on producers, the eradication of animal diseases, such as scrapie, is critical to ensuring the long-term sustainability of the sheep industry," federal chief veterinary officer Dr. Brian Evans said in CFIA's release.
Quarantine breaches "may put the livestock industry and the economy at risk," CFIA warned, adding that anyone who breaches a quarantine could be subject to prosecution under the federal Health of Animals Act.
Furthermore, any farm or other premises receiving these particular animals may also be subject to a quarantine and "further regulatory action," the agency added.
Scrapie is also a World Organization for Animal Health (OIE)-listed disease, CFIA said, thus the federal government has "international and trade obligations" to respond to any suspected cases.
"Although we sympathize with owners of affected flocks, Canada must deal effectively with this disease," Dr. Paula Menzies of the Small Ruminant Veterinarians of Ontario said in the CFIA release.
http://www.albertafarmexpress.ca/news/scrapie-quarantined-sheep-vanish-from-ont-farm/1001036775/
Saturday, February 27, 2010
FINAL REPORT OF THE TESTING OF THE BELGIAN (VERMONT) SHEEP February 27, 2010
IN SHORT ;
August 15, 2000
OIG case # NY-3399-56 REDACTED, VT
''Enclosed is OIG's notification that they have scheduled an investigation of the following individual. REDACTED is alleged to have provided possibly inaccurate test results involving diseased sheep. However, because the results were determined to be inconclusive, no actual violation was actually committed.''
snip...
PLEASE SEE FULL TEXT HERE ;
http://foiamadsheepmadrivervalley.blogspot.com/2010/02/final-report-of-testing-of-belgian.html
http://foiamadsheepmadrivervalley.blogspot.com/
atypical Nor-98 Scrapie has spread from coast to coast since first recognized in 2007. ...tss
Thursday, March 29, 2012
atypical Nor-98 Scrapie has spread from coast to coast in the USA 2012
NIAA Annual Conference April 11-14, 2011San Antonio, Texas
http://nor-98.blogspot.com/2012/03/atypical-nor-98-scrapie-has-spread-from.html
Wednesday, January 18, 2012
Selection of Distinct Strain Phenotypes in Mice Infected by Ovine Natural Scrapie Isolates Similar to CH1641 Experimental Scrapie
Journal of Neuropathology & Experimental Neurology:
February 2012 - Volume 71 - Issue 2 - p 140–147
http://transmissiblespongiformencephalopathy.blogspot.com/2012/01/selection-of-distinct-strain-phenotypes.html
Wednesday, February 16, 2011
IN CONFIDENCE
SCRAPIE TRANSMISSION TO CHIMPANZEES
IN CONFIDENCE
http://scrapie-usa.blogspot.com/2011/02/in-confidence-scrapie-transmission-to.html
Sunday, April 18, 2010
SCRAPIE AND ATYPICAL SCRAPIE TRANSMISSION STUDIES A REVIEW 2010
http://scrapie-usa.blogspot.com/2010/04/scrapie-and-atypical-scrapie.html
Monday, April 25, 2011
Experimental Oral Transmission of Atypical Scrapie to Sheep
Volume 17, Number 5-May 2011
http://nor-98.blogspot.com/2011/04/experimental-oral-transmission-of.html
Sunday, March 28, 2010
Nor-98 atypical Scrapie, atypical BSE, spontaneous TSE, trade policy, sound science ?
http://nor-98.blogspot.com/2010/03/nor-98-atypical-scrapie-atypical-bse.html
Monday, November 30, 2009
USDA AND OIE COLLABORATE TO EXCLUDE ATYPICAL SCRAPIE NOR-98 ANIMAL HEALTH CODE
http://nor-98.blogspot.com/2009/11/usda-and-oie-collaborate-to-exclude.html
I strenuously urge the USDA and the OIE et al to revoke the exemption of the legal global trading of atypical Nor-98 scrapie TSE. ...TSS
Friday, February 11, 2011
Atypical/Nor98 Scrapie Infectivity in Sheep Peripheral Tissues
http://nor-98.blogspot.com/2011/02/atypicalnor98-scrapie-infectivity-in.html
Sunday, March 11, 2012
APHIS Proposes New Bovine Spongiform Encephalopathy Import Regulations in Line with International Animal Health Standards Proposal Aims to Ensure Health of the U.S. Beef Herd, Assist in Negotiations
http://transmissiblespongiformencephalopathy.blogspot.com/2012/03/aphis-proposes-new-bovine-spongiform.html
TSS
Wednesday, April 4, 2012
Bovine Spongiform Encephalopathy; Importation of Bovines and Bovine Products APHIS-2008-0010-0008 RIN:0579-AC68
Family friend of John Gummer is killed by CJD aged 23 By ANDREW LEVY
Last updated at 19:16 11 October 2007
Elizabeth Smith: She learned on her 21st birthday that she had vCJD
A family friend of former Tory agriculture minister John Gummer has died from the human form of mad cow disease.
Elizabeth Smith died last week, more than two years after learning on her 21st birthday that she had new variant Creutzfeldt-Jakob disease.
Her father, retired vicar Roger Smith, is a friend of Mr Gummer, a former parishioner who famously attempted to allay fears about BSE in 1990 when he publicly fed a burger to his four-year-old daughter, Cordelia.
At the time, Mr Gummer said: "I can assure the public there is no cause for concern.
"The Government has taken all the advice it can from the experts. Their conclusion is that beef is perfectly safe."
University student Miss Smith quit her course days after the diagnosis in March 2005 and soon became so ill she needed round-the-clock treatment.
She was 23 when she died at her parents' home. Yesterday, they paid tribute to their "active and intelligent" daughter.
Mr Smith, of St Margaret South Elmham in Suffolk, said: "By the time she came home she had trouble swallowing and then couldn't swallow at all, so for the last two-and-a-half years she was fitted with a gastro-tube.
"After that the disease was remorseless in the way that it killed her off. She was unable to walk for the last two years of her life and couldn't speak or smile.
"Elizabeth had to be cared for 24 hours a day, seven days a week.
It's safe: John Gummer feeds his daughter Cordelia a burger
"She was more helpless for those last two years than when she was born - at least then she could move her arms and cry but by the end she couldn't even do that."
Describing her daughter's 21st birthday, Molly Smith said: "That was the worst time because we all had to cope with the fact that she was going to die.
"Elizabeth was clever, bright and intelligent. If she had been able to do her final exams she would have got a very good degree.
"She wanted to do primary school teaching and had a place on a postgraduate training course."
Miss Smith, who had a brother, Andrew, 39, passed four A-levels before going to Birmingham University in 2002 to read geography.
She first became ill in August 2004 but it was not until seven months later that she was told by doctors she had vCJD.
Mr Smith said yesterday that he was "99.9 per cent certain" that his daughter's illness had been caused by contaminated beef.
But he refused to blame Mr Gummer, saying the episode with the burger had not changed the way he viewed meat.
He added: "One of the few comforting thoughts is that almost certainly Elizabeth's degree of awareness in the last two years of her life was minimal. Some doctors would say that vCJD is far more painful to watch than suffer."
Miss Smith was the 162nd person to die from new variant CJD, which was first identified in 1996 after being linked to an outbreak of BSE (bovine spongiform encephalopathy) in cattle. vCJD slowly destroys the brain, giving it a sponge-like appearance.
It is thought to be caused by the build up in the brain of an abnormal form of the naturally occurring prion protein.
Most cases have developed as a result of eating infected meat, although five victims have been vegetarians. The disease has also been transmitted by blood transfusion and infected surgical equipment.
Mr Gummer was not available for comment yesterday.
http://www.dailymail.co.uk/health/article-487074/Family-friend-John-Gummer-killed-CJD-aged-23.html
GOVERNOR RICK PERRY TEXAS PHOTO OP
https://ichef.bbci.co.uk/news/304/media/images/59522000/jpg/_59522150_perry.jpg
Subject: Bovine Spongiform Encephalopathy; Importation of Bovines and Bovine Products APHIS-2008-0010-0008 RIN:0579-AC68
Comment from Terry Singeltary
Document ID: APHIS-2008-0010-0008 Document Type: Public Submission
This is comment on Proposed Rule: Bovine Spongiform Encephalopathy; Importation of Bovines and Bovine Products
Docket ID: APHIS-2008-0010 RIN:0579-AC68
Topics: No Topics associated with this document
View Document:
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Document Subtype: Public Comment
Status: Posted
Received Date: March 22 2012, at 12:00 AM Eastern Daylight Time
Date Posted: March 22 2012, at 12:00 AM Eastern Daylight Time
Comment Start Date: March 16 2012, at 12:00 AM Eastern Daylight Time
Comment Due Date: May 15 2012, at 11:59 PM Eastern Daylight Time
Tracking Number: 80fdd617
First Name: Terry
Middle Name: S.
Last Name: Singeltary
City: Bacliff
Country: United States
State or Province: TX
Organization Name: CJD TSE PRION
Submitter's Representative: CONSUMERS
Comment:
comment submission
Document ID APHIS-2008-0010-0001
Greetings USDA, OIE et al,
what a difference it makes with science, from one day to the next. i.e. that mad cow gold card the USA once held. up until that fateful day in December of 2003, the science of BSE was NO IMPORTS TO USA FROM BSE COUNTRY. what a difference a day makes$ now that the shoe is on the other foot, the USDA via the OIE, wants to change science again, just for trade $ I implore the OIE decision and policy makers, for the sake of the world, to refuse any status quo of the USA BSE risk assessment. if at al, the USA BSE GBR should be raise to BSE GBR IV, for the following reasons. North America is awash with many different TSE Prion strains, in many different species, and they are mutating and spreading. IF the OIE, and whatever policy makers, do anything but raise the risk factor for BSE in North America, they I would regard that to be highly suspicious. IN fact, it would be criminal in my opinion, because the OIE knows this, and to knowingly expose the rest of the world to this dangerous pathogen, would be ‘knowingly’ and ‘willfully’, just for the almighty dollar, once again. I warned the OIE about all this, including the risk factors for CWD, and the fact that the zoonosis potential was great, way back in 2002. THE OIE in collaboration with the USDA, made the legal trading of the atypical Nor-98 Scrapie a legal global commodity. yes, thanks to the OIE and the USDA et al, it’s now legal to trade the atypical Nor-98 Scrapie strain all around the globe. IF you let them, they will do the same thing with atypical BSE and CWD (both strains to date). This with science showing that indeed these TSE prion strains are transmissible. I strenuously urge the OIE et al to refuse any weakening to the USA trade protocols for the BSE TSE prion disease (all strains), and urge them to reclassify the USA with BSE GBR IV risk factor.
SEE REFERENCE SOURCES IN ATTACHMENTS
SEE Terry S. Singeltary Sr. Attachment WORD FILE ;
http://www.regulations.gov/#!documentDetail;D=APHIS-2008-0010-0008
http://www.regulations.gov/#!docketDetail;D=APHIS-2008-0010
Sunday, March 11, 2012
APHIS Proposes New Bovine Spongiform Encephalopathy Import Regulations in Line with International Animal Health Standards Proposal Aims to Ensure Health of the U.S. Beef Herd, Assist in Negotiations
http://transmissiblespongiformencephalopathy.blogspot.com/2012/03/aphis-proposes-new-bovine-spongiform.html
L-BSE, TME, AND SPORADIC CJD aka mad cow disease in North America
Moreover, transmission experiments to non-human primates suggest that some TSE agents in addition to Classical BSE prions in cattle (namely L-type Atypical BSE, Classical BSE in sheep, transmissible mink encephalopathy (TME) and chronic wasting disease (CWD) agents) might have zoonotic potential.
snip...
http://www.efsa.europa.eu/en/efsajournal/pub/e991.htm?emt=1
http://www.efsa.europa.eu/en/efsajournal/doc/e991.pdf
Thursday, August 12, 2010
Seven main threats for the future linked to prions
First threat
The TSE road map defining the evolution of European policy for protection against prion diseases is based on a certain numbers of hypotheses some of which may turn out to be erroneous. In particular, a form of BSE (called atypical Bovine Spongiform Encephalopathy), recently identified by systematic testing in aged cattle without clinical signs, may be the origin of classical BSE and thus potentially constitute a reservoir, which may be impossible to eradicate if a sporadic origin is confirmed.
***Also, a link is suspected between atypical BSE and some apparently sporadic cases of Creutzfeldt-Jakob disease in humans. These atypical BSE cases constitute an unforeseen first threat that could sharply modify the European approach to prion diseases.
Second threat
snip...
http://www.neuroprion.org/en/np-neuroprion.html
Saturday, June 25, 2011
Transmissibility of BSE-L and Cattle-Adapted TME Prion Strain to Cynomolgus Macaque
Emmanuel Comoy,1,† Valérie Durand,1 Evelyne Correia,1 Sophie Freire,1 Jürgen Richt,2 Justin Greenlee,3 Juan-Maria Torres,4 Paul Brown,1 Bob Hills5 and Jean-Philippe Deslys1
1Atomic Energy Commission; Fontenay-aux-Roses, France; 2Kansas State University; Manhattan, KS USA; 3USDA; Ames, IA USA; 4INIA; Madrid, Spain; 5Health Canada; Ottawa, ON Canada†Presenting author; Email: emmanuel.comoy@cea.fr
The epidemiology of Transmissible mink encephalopathy (TME) indicates an alimentary origin. Several inter-species transmission experiments have not succeeded in establishing with certainty any natural reservoir of this prion strain, although both ovine and bovine sources have been suspected. Cattle exposed to TME develop a spongiform encephalopathy that is distinct from classical Bovine Spongiform Encephalopathy (c-BSE).
Inoculation of c-BSE to cynomolgus macaque provided early evidence of a possible risk to humans, and remains an important model to define the risk of both primary (oral transmission from cattle to primate) and secondary (intravenous intra-species transmission) exposures. We have also evaluated the transmissibility of other cattle prion strains to macaques, including L- and H- atypical forms of BSE, namely BSE-L and BSE-H, and cattle-adapted TME.
BSE-L induced a neurological disease distinct from c-BSE. Peripheral exposures demonstrate the transmissibility of BSE-L by oral, intravenous, and intra-cerebral routes, with incubation periods similar to c-BSE. Cattle-adapted TME also induced a rapid disease in cynomolgus macaque. The clinical features, lesion profile, and biochemical signature of the induced disease was similar to the features observed in animals exposed to BSE-L, suggesting a link between the two prion strains. Secondary transmissions to a common host (transgenic mouse overexpressing bovine PrP) of cattle-TME and BSE-L before or after passage in primates induced diseases with similar incubation periods: like the c-BSE strain, these cattle strains maintained their distinctive features regardless of the donor species and passages.
If the link between TME and BSE-L is confirmed, our results would suggest that BSE-L in North America may have existed for decades, and highlight a possible preferential transmission of animal prion strains to primates after passage in cattle.
=====================end...tss====================
link url not available, please see PRION 2011 ;
http://www.prion2011.ca/files/PRION_2011_-_Posters_(May_5-11).pdf
Volume 13, Number 12–December 2007
Research
Phenotypic Similarity of Transmissible Mink Encephalopathy in Cattle and L-type Bovine Spongiform Encephalopathy in a Mouse Model
Thierry Baron,* Anna Bencsik,* Anne-Gaëlle Biacabe,* Eric Morignat,* andRichard A. Bessen†*Agence Française de Sécurité Sanitaire des Aliments–Lyon, Lyon, France; and†Montana State University, Bozeman, Montana, USA
Abstract
Transmissible mink encepholapathy (TME) is a foodborne transmissible spongiform encephalopathy (TSE) of ranch-raised mink; infection with a ruminant TSE has been proposed as the cause, but the precise origin of TME is unknown. To compare the phenotypes of each TSE, bovine-passaged TME isolate and 3 distinct natural bovine spongiform encephalopathy (BSE) agents (typical BSE, H-type BSE, and L-type BSE) were inoculated into an ovine transgenic mouse line (TgOvPrP4). Transgenic mice were susceptible to infection with bovine-passaged TME, typical BSE, and L-type BSE but not to H-type BSE. Based on survival periods, brain lesions profiles, disease-associated prion protein brain distribution, and biochemical properties of protease-resistant prion protein, typical BSE had a distint phenotype in ovine transgenic mice compared to L-type BSE and bovine TME.The similar phenotypic properties of L-type BSE and bovine TME in TgOvPrP4 mice suggest that L-type BSE is a much more likely candidate for the origin of TME than is typical BSE.
snip...
Conclusion
These studies provide experimental evidence that the Stetsonville TME agent is distinct from typical BSE but has phenotypic similarities to L-type BSE in TgOvPrP4 mice. Our conclusion is that L-type BSE is a more likely candidate for a bovine source of TME infection than typical BSE. In the scenario that a ruminant TSE is the source for TME infection in mink, this would be a second example of transmission of a TSE from ruminants to non-ruminants under natural conditions or farming practices in addition to transmission of typical BSE to humans, domestic cats, and exotic zoo animals(37). The potential importance of this finding is relevant to L-type BSE, which based on experimental transmission into humanized PrP transgenic mice and macaques, suggests that L-type BSE is more pathogenic for humans than typical BSE (24,38).
http://www.cdc.gov/eid/content/13/12/1887.htm?s_cid=eid1887_e
PLEASE NOTE *
Over the next 8-10 weeks, approximately 40% of all the adult mink on the farm died from TME.
snip...
The rancher was a ''dead stock'' feeder using mostly (>95%) downer or dead dairy cattle...
http://web.archive.org/web/20030516051623/http://www.bseinquiry.gov.uk/files/mb/m09/tab05.pdf
PLoS One. 2012; 7(2): e31449.
Published online 2012 February 21. doi: 10.1371/journal.pone.0031449
PMCID: PMC3283643
Infectivity in Skeletal Muscle of Cattle with Atypical Bovine Spongiform Encephalopathy
The present data offer novel information on the tropism of the BASE agent and highlight relevant public health issues. While the transmission barrier for classical BSE is high in most species, BASE prions are readily transmissible to a variety of mammals including non-human primates [11]–[13], [35]. Accordingly, the possibility of spreading of BASE prions through skeletal muscle to other species should be taken into account and evaluated in risk analysis studies.
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3283643/?tool=pubmed
Thursday, March 29, 2012
atypical Nor-98 Scrapie has spread from coast to coast in the USA 2012
NIAA Annual Conference April 11-14, 2011San Antonio, Texas
http://nor-98.blogspot.com/2012/03/atypical-nor-98-scrapie-has-spread-from.html
WHICH CAME FIRST, THE CART OR THE HORSE ???
Minnesota
CAPTIVE CWD CONFIRMED 2002
FREE RANGING CWD CONFIRMED 2011
http://wwwnc.cdc.gov/eid/article/18/3/11-0685-f1.htm
Colorado
Captive CWD discovered 1967
Free ranging CWD discovered 1981
PLEASE STUDY THIS MAP !
SEE CWD MAP, RELATE TO DATES OF GAME FARM INFECTION, TO DATE OF INFECTION RATE IN WILD, SURROUNDING SAID INFECTED GAME FARMS. ...TSS
http://wwwnc.cdc.gov/eid/article/18/3/11-0685-f1.htm
*** Chronic Wasting Disease CWD CDC REPORT MARCH 2012 ***
Saturday, February 18, 2012
Occurrence, Transmission, and Zoonotic Potential of Chronic Wasting Disease
CDC Volume 18, Number 3—March 2012
SNIP...
Long-term effects of CWD on cervid populations and ecosystems remain unclear as the disease continues to spread and prevalence increases. In captive herds, CWD might persist at high levels and lead to complete herd destruction in the absence of human culling. Epidemiologic modeling suggests the disease could have severe effects on free-ranging deer populations, depending on hunting policies and environmental persistence (8,9). CWD has been associated with large decreases in free-ranging mule deer populations in an area of high CWD prevalence (Boulder, Colorado, USA) (5).
SNIP...
Reasons for Caution There are several reasons for caution with respect to zoonotic and interspecies CWD transmission. First, there is strong evidence that distinct CWD strains exist (36). Prion strains are distinguished by varied incubation periods, clinical symptoms, PrPSc conformations, and CNS PrPSc depositions (3,32). Strains have been identified in other natural prion diseases, including scrapie, BSE, and CJD (3). Intraspecies and interspecies transmission of prions from CWD-positive deer and elk isolates resulted in identification of >2 strains of CWD in rodent models (36), indicating that CWD strains likely exist in cervids. However, nothing is currently known about natural distribution and prevalence of CWD strains. Currently, host range and pathogenicity vary with prion strain (28,37). Therefore, zoonotic potential of CWD may also vary with CWD strain. In addition, diversity in host (cervid) and target (e.g., human) genotypes further complicates definitive findings of zoonotic and interspecies transmission potentials of CWD. Intraspecies and interspecies passage of the CWD agent may also increase the risk for zoonotic CWD transmission. The CWD prion agent is undergoing serial passage naturally as the disease continues to emerge. In vitro and in vivo intraspecies transmission of the CWD agent yields PrPSc with an increased capacity to convert human PrPc to PrPSc (30). Interspecies prion transmission can alter CWD host range (38) and yield multiple novel prion strains (3,28). The potential for interspecies CWD transmission (by cohabitating mammals) will only increase as the disease spreads and CWD prions continue to be shed into the environment. This environmental passage itself may alter CWD prions or exert selective pressures on CWD strain mixtures by interactions with soil, which are known to vary with prion strain (25), or exposure to environmental or gut degradation. Given that prion disease in humans can be difficult to diagnose and the asymptomatic incubation period can last decades, continued research, epidemiologic surveillance, and caution in handling risky material remain prudent as CWD continues to spread and the opportunity for interspecies transmission increases. Otherwise, similar to what occurred in the United Kingdom after detection of variant CJD and its subsequent link to BSE, years of prevention could be lost if zoonotic transmission of CWD is subsequently identified, SNIP...SEE FULL TEXT ;
*** Chronic Wasting Disease CWD CDC REPORT MARCH 2012 ***
Saturday, February 18, 2012
Occurrence, Transmission, and Zoonotic Potential of Chronic Wasting Disease
CDC Volume 18, Number 3—March 2012
http://wwwnc.cdc.gov/eid/article/18/3/11-0685_article.htm
see much more here ;
http://chronic-wasting-disease.blogspot.com/2012/02/occurrence-transmission-and-zoonotic.html
Sunday, January 22, 2012
Chronic Wasting Disease CWD cervids interspecies transmission
http://chronic-wasting-disease.blogspot.com/2012/01/chronic-wasting-disease-cwd-cervids.html
Thursday, January 26, 2012
The Risk of Prion Zoonoses
Science 27 January 2012: Vol. 335 no. 6067 pp. 411-413 DOI: 10.1126/science.1218167
http://transmissiblespongiformencephalopathy.blogspot.com/2012/01/risk-of-prion-zoonoses.html
Thursday, January 26, 2012
Facilitated Cross-Species Transmission of Prions in Extraneural Tissue
Science 27 January 2012: Vol. 335 no. 6067 pp. 472-475 DOI: 10.1126/science.1215659
http://transmissiblespongiformencephalopathy.blogspot.com/2012/01/facilitated-cross-species-transmission.html
CJD9/10022
October 1994
Mr R.N. Elmhirst Chairman British Deer Farmers Association Holly Lodge Spencers Lane BerksWell Coventry CV7 7BZ
Dear Mr Elmhirst,
CREUTZFELDT-JAKOB DISEASE (CJD) SURVEILLANCE UNIT REPORT
Thank you for your recent letter concerning the publication of the third annual report from the CJD Surveillance Unit. I am sorry that you are dissatisfied with the way in which this report was published.
The Surveillance Unit is a completely independant outside body and the Department of Health is committed to publishing their reports as soon as they become available. In the circumstances it is not the practice to circulate the report for comment since the findings of the report would not be amended. In future we can ensure that the British Deer Farmers Association receives a copy of the report in advance of publication.
The Chief Medical Officer has undertaken to keep the public fully informed of the results of any research in respect of CJD. This report was entirely the work of the unit and was produced completely independantly of the the Department.
The statistical results reqarding the consumption of venison was put into perspective in the body of the report and was not mentioned at all in the press release. Media attention regarding this report was low key but gave a realistic presentation of the statistical findings of the Unit. This approach to publication was successful in that consumption of venison was highlighted only once by the media ie. in the News at one television proqramme.
I believe that a further statement about the report, or indeed statistical links between CJD and consumption of venison, would increase, and quite possibly give damaging credence, to the whole issue. From the low key media reports of which I am aware it seems unlikely that venison consumption will suffer adversely, if at all.
http://web.archive.org/web/20030511010117/http://www.bseinquiry.gov.uk/files/yb/1994/10/00003001.pdf
THIRD CJD REPORT UK 1994
snip...
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. These associations were largely unchanged when attention was restricted to pairs with data obtained from relatives. ...
http://www.cjd.ed.ac.uk/Archive%20reports/report3.pdf
Saturday, March 10, 2012
Enhanced Surveillance Strategies for Detecting and Monitoring Chronic Wasting Disease in Free-Ranging Cervids Open-File Report 2012–1036 National Wildlife Health Center
Open-File Report 2012–1036
http://chronic-wasting-disease.blogspot.com/2012/03/enhanced-surveillance-strategies-for.html
a few things to consider please. one, CWD has already been transmitted to many cattle in the lab (86% in one study). the oral route would have a much longer incubation period, but we already know that CWD will transmit back to cervids via the oral route, very efficiently. the threat of spreading CWD via close contact, like at feeding grounds is great. every bit of science to date shows this. so to congregate deer together by unnatural means is not smart in my opinion. another fear has come to pass as well, another strain of CWD, yes a second strain. and just recently science has shown that a natural case of BSE has been transmitted to a GOAT. These findings demonstrate that when CWD is directly inoculated into the brain of cattle, 86% of inoculated cattle develop clinical signs of the disease.
http://www.ars.usda.gov/research/publications/publications.htm?seq_no_115=194089
Thursday, February 09, 2012
50 GAME FARMS IN USA INFECTED WITH CHRONIC WASTING DISEASE
http://chronic-wasting-disease.blogspot.com/2012/02/50-game-farms-to-date-in-usa-infected.html
and when these game farms claim they are testing, and everything is o.k., think again...
Saturday, February 04, 2012
Wisconsin 16 age limit on testing dead deer Game Farm CWD Testing Protocol Needs To Be Revised
http://chronic-wasting-disease.blogspot.com/2012/02/wisconsin-16-age-limit-on-testing-dead.html
Saturday, November 6, 2010
TAFS1 Position Paper on Position Paper on Relaxation of the Feed Ban in the EU
Berne, 2010 TAFS INTERNATIONAL FORUM FOR TRANSMISSIBLE ANIMAL DISEASES AND FOOD SAFETY a non-profit Swiss Foundation
http://madcowfeed.blogspot.com/2010/11/tafs1-position-paper-on-position-paper.html
Archive Number 20101206.4364 Published Date 06-DEC-2010 Subject PRO/AH/EDR>
Prion disease update 2010 (11) PRION DISEASE UPDATE 2010 (11)
http://www.promedmail.org/direct.php?id=20101206.4364
> > > Ackerman says downed cattle are 50 times more likely to have mad cow disease (also known as Bovine Spongiform Encephalopathy, or BSE) than ambulatory cattle that are suspected of having BSE. Of the 20 confirmed cases of mad cow disease in North America since 1993, at least 16 have involved downer cattle, he said. < < <
don’t forget the children...
PLEASE be aware, for 4 years, the USDA fed our children all across the Nation (including TEXAS) dead stock downer cows, the most high risk cattle for BSE aka mad cow disease and other dangerous pathogens.
who will watch our children for CJD for the next 5+ decades ???
WAS your child exposed to mad cow disease via the NSLP ???
SCHOOL LUNCH PROGRAM FROM DOWNER CATTLE UPDATE
http://downercattle.blogspot.com/2009/05/who-will-watch-children.html
http://downercattle.blogspot.com/
DID YOUR CHILD CONSUME SOME OF THESE DEAD STOCK DOWNER COWS, THE MOST HIGH RISK FOR MAD COW DISEASE ???
you can check and see here ;
http://www.fns.usda.gov/fns/safety/pdf/Hallmark-Westland_byState.pdf
Saturday, March 5, 2011
MAD COW ATYPICAL CJD PRION TSE CASES WITH CLASSIFICATIONS PENDING ON THE RISE IN NORTH AMERICA
http://transmissiblespongiformencephalopathy.blogspot.com/2011/03/mad-cow-atypical-cjd-prion-tse-cases.html
Sunday, February 12, 2012
National Prion Disease Pathology Surveillance Center Cases Examined1 (August 19, 2011) including Texas
http://transmissiblespongiformencephalopathy.blogspot.com/2012/02/national-prion-disease-pathology.html
pink slime and a ship of fools, with Governor Rick Perry at the helm.
john gummer of England, force fed his daughter mad cow beef. a few years later, a young friend of theirs (23) died from mad cow disease. NOW, Governor Rick Perry, shows he is as big a fool as John Gummer.
http://media.kansascity.com/smedia/2012/03/29/21/01/MiPpi.SlMa.81.jpg
http://i.dailymail.co.uk/i/pix/2009/12/30/article-0-01F258B0000004B0-450_468x286.jpg
see more on this sad sad saga here ;
Wednesday, March 14, 2012
PINK SLIME, MRM’s, BSE AKA MAD COW DISEASE, AND THE USDA NSLP
http://madcowusda.blogspot.com/2012/03/pink-slime-mrms-bse-aka-mad-cow-disease.html
Sunday, August 28, 2011
Rick Perry, Texas, BSE aka mad cow disease, CJD, and 12 years of lies there from
http://sciencebushwhacked.blogspot.com/2011/08/rick-perry-texas-bse-aka-mad-cow.html
BY the way, ammonia treated beef DOES NOT KILL MAD COW DISEASE !!!
MOM DOD 12-14-97 Heidenhain Variant Creutzfeldt Jakob Disease ‘confirmed’ TEXAS, YEARS OF RICK PERRY BEING TEXAS AGRICULTURE COMMISSIONER, THEN ON TO GOVERNOR OF TEXAS, WHERE TWO MAD COWS WERE COVERED UP under Perry’s watch, ONE SUCCESSFULLY, AND ONE THAT TOOK AN ACT OF CONGRESS and 7 MONTHS TO FINALLY CONFIRM via WEYBRIDGE ENGLAND.
layperson
Terry S. Singeltary Sr.
Bacliff, Texas USA 77518
flounder9@verizon.net
Monday, March 19, 2012
Infectivity in Skeletal Muscle of Cattle with Atypical Bovine Spongiform Encephalopathy
PLoS One. 2012; 7(2): e31449.
Published online 2012 February 21. doi: 10.1371/journal.pone.0031449
PMCID: PMC3283643
Copyright Suardi et al. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.
Infectivity in Skeletal Muscle of Cattle with Atypical Bovine Spongiform Encephalopathy
Silvia Suardi,#1 Chiara Vimercati,#1 Cristina Casalone,#2 Daniela Gelmetti,3 Cristiano Corona,2 Barbara Iulini,2 Maria Mazza,2 Guerino Lombardi,3 Fabio Moda,1 Margherita Ruggerone,1 Ilaria Campagnani,1 Elena Piccoli,1 Marcella Catania,1 Martin H. Groschup,4 Anne Balkema-Buschmann,4 Maria Caramelli,2 Salvatore Monaco,5 Gianluigi Zanusso,5 and Fabrizio Tagliavini1*
1Instituto Di Ricoveroe Cura a Carattere Scientifico (IRCCS), Foundation “Carlo Besta” Neurological Institute, Milano, Italy
2Istituto Zooprofilattico Sperimentale del Piemonte, Liguria e Valle d'Aosta, Torino, Italy
3Istituto Zooprofilattico Sperimentale della Lombardia ed Emilia Romagna, Brescia, Italy
4Friedrich-Loeffler-Institut, Greifswald, Insel Riems, Germany
5Policlinico G.B. Rossi, University of Verona, Verona, Italy
Jason Bartz, Editor
Creighton University, United States of America
#Contributed equally.
* E-mail: ftagliavini@istituto-besta.it
Conceived and designed the experiments: SS CV C. Casalone DG C. Corona GL SM M. Caramelli GZ FT. Performed the experiments: SS CV DG C. Corona BI MM FM MR IC EP M. Catania. Analyzed the data: SS CV C. Casalone DG C. Corona GL SM MHG AB M. Caramelli GZ FT. Contributed reagents/materials/analysis tools: SS CV C. Casalone DG C. Corona GL SM MHG AB M. Caramelli GZ FT. Wrote the paper: SS CV C. Casalone DG C. Corona GL SM MHG AB M.Caramelli GZ FT.
Received December 13, 2011; Accepted January 8, 2012.
Abstract
The amyloidotic form of bovine spongiform encephalopathy (BSE) termed BASE is caused by a prion strain whose biological properties differ from those of typical BSE, resulting in a clinically and pathologically distinct phenotype. Whether peripheral tissues of BASE-affected cattle contain infectivity is unknown. This is a critical issue since the BASE prion is readily transmissible to a variety of hosts including primates, suggesting that humans may be susceptible. We carried out bioassays in transgenic mice overexpressing bovine PrP (Tgbov XV) and found infectivity in a variety of skeletal muscles from cattle with natural and experimental BASE. Noteworthy, all BASE muscles used for inoculation transmitted disease, although the attack rate differed between experimental and natural cases (~70% versus ~10%, respectively). This difference was likely related to different prion titers, possibly due to different stages of disease in the two conditions, i.e. terminal stage in experimental BASE and pre-symptomatic stage in natural BASE. The neuropathological phenotype and PrPres type were consistent in all affected mice and matched those of Tgbov XV mice infected with brain homogenate from natural BASE. The immunohistochemical analysis of skeletal muscles from cattle with natural and experimental BASE showed the presence of abnormal prion protein deposits within muscle fibers. Conversely, Tgbov XV mice challenged with lymphoid tissue and kidney from natural and experimental BASE did not develop disease. The novel information on the neuromuscular tropism of the BASE strain, efficiently overcoming species barriers, underlines the relevance of maintaining an active surveillance.
snip...
Introduction
In 2004 an atypical form of bovine spongiform encephalopathy (BSE) termed BASE or BSE-L was identified in Italy through active surveillance [1] and subsequently recognized in different European countries, North America, Canada and Japan [1]–[6]. BASE affects relatively old cattle (all cases were older than 9 years) and differs from BSE with regard to the neuropathological phenotype, the biochemical profile of the disease-associated prion protein (PrPSc) and the biological properties of the agent strain [1], [3], [7]. The neuropathological hallmark of BASE is the presence of PrP amyloid plaques and the preferential involvement of olfactory areas, hippocampus and thalamus with a relatively low involvement of the brainstem, as opposed to classical BSE. The molecular signature of BASE is a PrPSc type distinguished by a protease-resistant core (PrPres) of lower molecular mass than BSE-PrPSc, with predominance of the monoglycosylated protein band by western immunoblot.
Intra-species transmission revealed that the clinical picture of BASE differs substantially from that of BSE, being characterized by mental dullness and amyotrophy rather than hyper-reactivity and aggressiveness [8], [9]. As a consequence, the recognition of BASE in vivo can be difficult and may represent a major challenge for passive surveillance. Transmission studies to transgenic mice overexpressing bovine PrP (Tgbov mice) showed that the BASE strain is more aggressive than the BSE strain [10]. Furthermore, Tg mice overexpressing human PrP as well as non-human primates are more susceptible to infection with BASE than with BSE [11]–[14]. Overall these data raise concern about the potential risk of transmission of BASE to humans and it is urgent to determine the presence and distribution of infectivity in peripheral tissues of BASE-affected cattle. To investigate this issue, we inoculated Tgbov mice with different peripheral tissues from experimentally and naturally BASE-affected cattle and found that various skeletal muscles contained infectivity and PrP-immunoreactive deposits within individual fibers.
snip...
Discussion
This is the first report on the occurrence and distribution of infectivity in peripheral tissues of BASE-affected cattle. We found that different skeletal muscles (i.e., M. longissimus dorsi, M. intercostalis and M. gluteus) from experimental and natural BASE carried infectivity, whereas spleen, cervical lymph node and kidney did not, as highly susceptible Tgbov XV mice did not develop disease up to 850 days after challenge. Noteworthy, all BASE muscles used for inoculation transmitted disease, although the attack rate differed between experimental and natural cases. This difference is likely related to different prion titers in the two conditions, possibly due to different stages of disease. In fact, the experimentally infected cattle was sacrificed at the terminal stage when clinical signs were severe [8], while the cattle with natural BASE was identified through active surveillance at a pre-symptomatic stage.
Infectivity in skeletal muscles has been detected in various natural and experimental prion diseases, including sheep affected by classical and Nor/98 atypical scrapie [17], deer with chronic wasting disease [18] and rodent models of scrapie [19]. A large pathogenesis study on BSE-infected cattle in the UK failed to detect infectivity in muscle tissue at any time during the course of the disease by inoculation of inbred mice [20]. However, in a subsequent study with Tgbov XV mice, M. semitendinosus from a field case of clinically symptomatic BSE transmitted disease to one out of ten inoculated rodents. This low amount of infectivity was tentatively related to the terminal nerve fibres [21].
A major clinical feature of cattle experimentally infected with BASE is amyotrophy, as a result of motor neuron disease [8]. Although we were unable to detect PrPSc in the peripheral nerves of infected cattle and follow the kinetics of PrP spreading through the neural pathway, a study of intra-species transmission of a Japanese case of BASE showed that PrPres was first detectable by immunoblot in the nerve roots and subsequently in the peripheral nerves [22]. PrPres deposition in skeletal muscles has been found in a variety of prion diseases, including experimental scrapie in hamsters [23], natural scrapie in sheep [24], and mouse and primate models of BSE and CJD [25]–[27]. These reports agree that PrPres in muscle tissue is associated with terminal nerve endings.
In our study, immunohistochemistry showed the presence of amorphous or granular, small PrP deposits in different skeletal muscles from both experimental and natural BASE-affected cattle. PrP aggregates were found in isolated muscle fibers with a scattered distribution which was at variance with the topology of PrPres reported in the previous studies [23], [24], [26], [27]. Noteworthy, identical results were obtained using two different fixatives and immunostaining protocols. Since PrPC is expressed in bovine muscles and skeletal muscles are intrinsically capable of propagating prions [19], [28], we argue that PrPSc deposition in BASE muscles might be the result of a primary PrP replication through a neural-independent pathway. This possibility has been previously considered in a patient affected by sporadic CJD and inclusion body myositis where PrPC to PrPSc conversion occurred in skeletal muscle [29].
The limited number and irregular distribution of PrP positive fibers within a muscle sample accounts for the absence of a detectable PrPres signal on Western blot. The inhomogeneous distribution of PrPres and infectivity among different muscles of the same animal and within the same muscle has been previously observed in mice and in primates infected with different prion strains. These findings have been tentatively related to biochemical differences in skeletal muscles of different body regions and/or number of nerve endings [19], [25], [26].
In the present study, Tgbov XV mice challenged with BASE muscles reproduced the monoglycosylated-dominant PrPres type of BASE as well as a histopathological lesion profile and pattern of PrPres deposition that matched those observed in mice challenged with BASE brain. These findings indicate that, in Tgbov XV mice, muscle and brain tissues maintain the same biological properties of the BASE strain.
Although serial transmission studies in Tgbov XV mice showed that the BASE prion is capable to replicate in the spleen converting, in part, into a BSE-like strain (personal observation), we did not detect infectivity in lymph node and spleen from cattle with experimental and natural BASE. This observation opposes the possibility that the BASE agent might re-circulate, potentially as BSE-like strain, in different hosts. Kidney is an edible organ and infectivity found in urine is kidney-associated [30]. Lack of transmission from kidney of natural and experimental BASE is an important issue, since infectivity in kidney has been demonstrated by bioassay in human prion diseases [31], and PrPSc has been observed in kidney of scrapie infected sheep and CWD affected deer [32]–[34].
The present data offer novel information on the tropism of the BASE agent and highlight relevant public health issues. While the transmission barrier for classical BSE is high in most species, BASE prions are readily transmissible to a variety of mammals including non-human primates [11]–[13], [35]. Accordingly, the possibility of spreading of BASE prions through skeletal muscle to other species should be taken into account and evaluated in risk analysis studies.
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3283643/?tool=pubmed
Saturday, June 25, 2011
Transmissibility of BSE-L and Cattle-Adapted TME Prion Strain to Cynomolgus Macaque
"BSE-L in North America may have existed for decades"
http://transmissiblespongiformencephalopathy.blogspot.com/2011/06/transmissibility-of-bse-l-and-cattle.html
Over the next 8-10 weeks, approximately 40% of all the adult mink on the farm died from TME.
snip...
The rancher was a ''dead stock'' feeder using mostly (>95%) downer or dead dairy cattle...
http://web.archive.org/web/20030516051623/http://www.bseinquiry.gov.uk/files/mb/m09/tab05.pdf
Monday, October 10, 2011
EFSA Journal 2011 The European Response to BSE: A Success Story
snip...
EFSA and the European Centre for Disease Prevention and Control (ECDC) recently delivered a scientific opinion on any possible epidemiological or molecular association between TSEs in animals and humans (EFSA Panel on Biological Hazards (BIOHAZ) and ECDC, 2011). This opinion confirmed Classical BSE prions as the only TSE agents demonstrated to be zoonotic so far but the possibility that a small proportion of human cases so far classified as "sporadic" CJD are of zoonotic origin could not be excluded. Moreover, transmission experiments to non-human primates suggest that some TSE agents in addition to Classical BSE prions in cattle (namely L-type Atypical BSE, Classical BSE in sheep, transmissible mink encephalopathy (TME) and chronic wasting disease (CWD) agents) might have zoonotic potential.
snip...
http://www.efsa.europa.eu/en/efsajournal/pub/e991.htm?emt=1
http://www.efsa.europa.eu/en/efsajournal/doc/e991.pdf
see follow-up here about North America BSE Mad Cow TSE prion risk factors, and the ever emerging strains of Transmissible Spongiform Encephalopathy in many species here in the USA, including humans ;
http://transmissiblespongiformencephalopathy.blogspot.com/2011/10/efsa-journal-2011-european-response-to.html
Thursday, August 12, 2010
Seven main threats for the future linked to prions
First threat
The TSE road map defining the evolution of European policy for protection against prion diseases is based on a certain numbers of hypotheses some of which may turn out to be erroneous. In particular, a form of BSE (called atypical Bovine Spongiform Encephalopathy), recently identified by systematic testing in aged cattle without clinical signs, may be the origin of classical BSE and thus potentially constitute a reservoir, which may be impossible to eradicate if a sporadic origin is confirmed.
***Also, a link is suspected between atypical BSE and some apparently sporadic cases of Creutzfeldt-Jakob disease in humans. These atypical BSE cases constitute an unforeseen first threat that could sharply modify the European approach to prion diseases.
Second threat
snip...
http://www.neuroprion.org/en/np-neuroprion.html
Rural and Regional Affairs and Transport References Committee
The possible impacts and consequences for public health, trade and agriculture of the Government's decision to relax import restrictions on beef Final report June 2010
2.65 At its hearing on 14 May 2010, the committee heard evidence from Dr Alan Fahey who has recently submitted a thesis on the clinical neuropsychiatric, epidemiological and diagnostic features of Creutzfeldt-Jakob disease.48 Dr Fahey told the committee of his concerns regarding the lengthy incubation period for transmissible spongiform encephalopathies, the inadequacy of current tests and the limited nature of our current understanding of this group of diseases.49
2.66 Dr Fahey also told the committee that in the last two years a link has been established between forms of atypical CJD and atypical BSE. Dr Fahey said that: They now believe that those atypical BSEs overseas are in fact causing sporadic Creutzfeldt-Jakob disease. They were not sure if it was due to mad sheep disease or a different form. If you look in the textbooks it looks like this is just arising by itself. But in my research I have a summary of a document which states that there has never been any proof that sporadic Creutzfeldt-Jakob disease has arisen de novo-has arisen of itself. There is no proof of that. The recent research is that in fact it is due to atypical forms of mad cow disease which have been found across Europe, have been found in America and have been found in Asia. These atypical forms of mad cow disease typically have even longer incubation periods than the classical mad cow disease.50
http://www.aph.gov.au/senate/committee/rrat_ctte/mad_cows/report/report.pdf
Atypical BSE in Cattle
To date the OIE/WAHO assumes that the human and animal health standards set out in the BSE chapter for classical BSE (C-Type) applies to all forms of BSE which include the H-type and L-type atypical forms. This assumption is scientifically not completely justified and accumulating evidence suggests that this may in fact not be the case. Molecular characterization and the spatial distribution pattern of histopathologic lesions and immunohistochemistry (IHC) signals are used to identify and characterize atypical BSE. Both the L-type and H-type atypical cases display significant differences in the conformation and spatial accumulation of the disease associated prion protein (PrPSc) in brains of afflicted cattle. Transmission studies in bovine transgenic and wild type mouse models support that the atypical BSE types might be unique strains because they have different incubation times and lesion profiles when compared to C-type BSE. When L-type BSE was inoculated into ovine transgenic mice and Syrian hamster the resulting molecular fingerprint had changed, either in the first or a subsequent passage, from L-type into C-type BSE.
In addition, non-human primates are specifically susceptible for atypical BSE as demonstrated by an approximately 50% shortened incubation time for L-type BSE as compared to C-type. Considering the current scientific information available, it cannot be assumed that these different BSE types pose the same human health risks as C-type BSE or that these risks are mitigated by the same protective measures.
This study will contribute to a correct definition of specified risk material (SRM) in atypical BSE. The incumbent of this position will develop new and transfer existing, ultra-sensitive methods for the detection of atypical BSE in tissue of experimentally infected cattle.
http://www.prionetcanada.ca/detail.aspx?menu=5&dt=293380&app=93&cat1=387&tp=20&lk=no&cat2
When L-type BSE was inoculated into ovine transgenic mice and Syrian hamster the resulting molecular fingerprint had changed, either in the first or a subsequent passage, from L-type into C-type BSE. In addition, non-human primates are specifically susceptible for atypical BSE as demonstrated by an approximately 50% shortened incubation time for L-type BSE as compared to C-type. Considering the current scientific information available, it cannot be assumed that these different BSE types pose the same human health risks as C-type BSE or that these risks are mitigated by the same protective measures.
This study will contribute to a correct definition of specified risk material (SRM) in atypical BSE. The incumbent of this position will develop new and transfer existing, ultra-sensitive methods for the detection of atypical BSE in tissue of experimentally infected cattle.
http://www.prionetcanada.ca/detail.aspx?menu=5&dt=293380&app=93&cat1=387&tp=20&lk=no&cat2
P.4.23
Transmission of atypical BSE in humanized mouse models
Liuting Qing1, Wenquan Zou1, Cristina Casalone2, Martin Groschup3, Miroslaw Polak4, Maria Caramelli2, Pierluigi Gambetti1, Juergen Richt5, Qingzhong Kong1 1Case Western Reserve University, USA; 2Instituto Zooprofilattico Sperimentale, Italy; 3Friedrich-Loeffler-Institut, Germany; 4National Veterinary Research Institute, Poland; 5Kansas State University (Previously at USDA National Animal Disease Center), USA
Background: Classical BSE is a world-wide prion disease in cattle, and the classical BSE strain (BSE-C) has led to over 200 cases of clinical human infection (variant CJD). Atypical BSE cases have been discovered in three continents since 2004; they include the L-type (also named BASE), the H-type, and the first reported case of naturally occurring BSE with mutated bovine PRNP (termed BSE-M). The public health risks posed by atypical BSE were largely undefined.
Objectives: To investigate these atypical BSE types in terms of their transmissibility and phenotypes in humanized mice. Methods: Transgenic mice expressing human PrP were inoculated with several classical (C-type) and atypical (L-, H-, or Mtype) BSE isolates, and the transmission rate, incubation time, characteristics and distribution of PrPSc, symptoms, and histopathology were or will be examined and compared.
Results: Sixty percent of BASE-inoculated humanized mice became infected with minimal spongiosis and an average incubation time of 20-22 months, whereas only one of the C-type BSE-inoculated mice developed prion disease after more than 2 years. Protease-resistant PrPSc in BASE-infected humanized Tg mouse brains was biochemically different from bovine BASE or sCJD. PrPSc was also detected in the spleen of 22% of BASE-infected humanized mice, but not in those infected with sCJD. Secondary transmission of BASE in the humanized mice led to a small reduction in incubation time.*** The atypical BSE-H strain is also transmissible with distinct phenotypes in the humanized mice, but no BSE-M transmission has been observed so far.
Discussion: Our results demonstrate that BASE is more virulent than classical BSE, has a lymphotropic phenotype, and displays a modest transmission barrier in our humanized mice. BSE-H is also transmissible in our humanized Tg mice. The possibility of more than two atypical BSE strains will be discussed.
Supported by NINDS NS052319, NIA AG14359, and NIH AI 77774.
http://www.prion2009.com/sites/default/files/Prion2009_Book_of_Abstracts.pdf
P26 TRANSMISSION OF ATYPICAL BOVINE SPONGIFORM ENCEPHALOPATHY (BSE) IN HUMANIZED MOUSE MODELS
Liuting Qing1, Fusong Chen1, Michael Payne1, Wenquan Zou1, Cristina Casalone2, Martin Groschup3, Miroslaw Polak4, Maria Caramelli2, Pierluigi Gambetti1, Juergen Richt5*, and Qingzhong Kong1 1Department of Pathology, Case Western Reserve University, Cleveland, OH 44106, USA; 2CEA, Istituto Zooprofilattico Sperimentale, Italy; 3Friedrich-Loeffler-Institut, Germany; 4National Veterinary Research Institute, Poland; 5Kansas State University, Diagnostic Medicine/Pathobiology Department, Manhattan, KS 66506, USA. *Previous address: USDA National Animal Disease Center, Ames, IA 50010, USA
Classical BSE is a world-wide prion disease in cattle, and the classical BSE strain (BSE-C) has led to over 200 cases of clinical human infection (variant CJD). Two atypical BSE strains, BSE-L (also named BASE) and BSE-H, have been discovered in three continents since 2004. The first case of naturally occurring BSE with mutated bovine PrP gene (termed BSE-M) was also found in 2006 in the USA. The transmissibility and phenotypes of these atypical BSE strains/isolates in humans were unknown. We have inoculated humanized transgenic mice with classical and atypical BSE strains (BSE-C, BSE-L, BSE-H) and the BSE-M isolate. We have found that the atypical BSE-L strain is much more virulent than the classical BSE-C.*** The atypical BSE-H strain is also transmissible in the humanized transgenic mice with distinct phenotype, but no transmission has been observed for the BSE-M isolate so far.
III International Symposium on THE NEW PRION BIOLOGY: BASIC SCIENCE, DIAGNOSIS AND THERAPY 2 - 4 APRIL 2009, VENEZIA (ITALY)
http://www.istitutoveneto.it/prion_09/Abstracts_09.pdf
I ask Professor Kong ;
Thursday, December 04, 2008 3:37 PM Subject: RE: re--Chronic Wating Disease (CWD) and Bovine Spongiform Encephalopathies (BSE): Public Health Risk Assessment
''IS the h-BSE more virulent than typical BSE as well, or the same as cBSE, or less virulent than cBSE? just curious.....''
Professor Kong reply ;
.....snip
''As to the H-BSE, we do not have sufficient data to say one way or another, but we have found that H-BSE can infect humans. I hope we could publish these data once the study is complete. Thanks for your interest.''
Best regards, Qingzhong Kong, PhD Associate Professor Department of Pathology Case Western Reserve University Cleveland, OH 44106 USA
END...TSS
Thursday, December 04, 2008 2:37 PM
"we have found that H-BSE can infect humans."
personal communication with Professor Kong. ...TSS
BSE-H is also transmissible in our humanized Tg mice.
The possibility of more than two atypical BSE strains will be discussed.
Supported by NINDS NS052319, NIA AG14359, and NIH AI 77774.
http://www.prion2009.com/sites/default/files/Prion2009_Book_of_Abstracts.pdf
http://transmissiblespongiformencephalopathy.blogspot.com/2011/06/experimental-h-type-bovine-spongiform.html
Tuesday, July 14, 2009 U.S.
Emergency Bovine Spongiform Encephalopathy Response Plan Summary and BSE Red Book
Date: February 14, 2000 at 8:56 am PST
WHERE did we go wrong $$$
http://madcowtesting.blogspot.com/2009/07/us-emergency-bovine-spongiform.html
LET'S take a closer look at this new prionpathy or prionopathy, and then let's look at the g-h-BSEalabama mad cow. This new prionopathy in humans? the genetic makeup is IDENTICAL to the g-h-BSEalabama mad cow, the only _documented_ mad cow in the world to date like this, ......wait, it get's better. this new prionpathy is killing young and old humans, with LONG DURATION from onset of symptoms to death, and the symptoms are very similar to nvCJD victims, OH, and the plaques are very similar in some cases too, bbbut, it's not related to the g-h-BSEalabama cow, WAIT NOW, it gets even better, the new human prionpathy that they claim is a genetic TSE, has no relation to any gene mutation in that family. daaa, ya think it could be related to that mad cow with the same genetic make-up ??? there were literally tons and tons of banned mad cow protein in Alabama in commerce, and none of it transmitted to cows, and the cows to humans there from ??? r i g h t $$$ ALABAMA MAD COW g-h-BSEalabama In this study, we identified a novel mutation in the bovine prion protein gene (Prnp), called E211K, of a confirmed BSE positive cow from Alabama, United States of America. This mutation is identical to the E200K pathogenic mutation found in humans with a genetic form of CJD. This finding represents the first report of a confirmed case of BSE with a potential pathogenic mutation within the bovine Prnp gene. We hypothesize that the bovine Prnp E211K mutation most likely has caused BSE in "the approximately 10-year-old cow" carrying the E221K mutation.
http://www.plospathogens.org/article/info%3Adoi%2F10.1371%2Fjournal.ppat.1000156
http://www.plospathogens.org/article/fetchObjectAttachment.action?uri=info%3Adoi%2F10.1371%2Fjournal.ppat.1000156&representation=PDF
Saturday, August 14, 2010
BSE Case Associated with Prion Protein Gene Mutation (g-h-BSEalabama) and VPSPr PRIONPATHY (see mad cow feed in COMMERCE IN ALABAMA...TSS)
http://prionpathy.blogspot.com/2010/08/bse-case-associated-with-prion-protein.html
her healthy calf also carried the mutation (J. A. Richt and S. M. Hall PLoS Pathog. 4, e1000156; 2008).
This raises the possibility that the disease could occasionally be genetic in origin. Indeed, the report of the UK BSE Inquiry in 2000 suggested that the UK epidemic had most likely originated from such a mutation and argued against the scrapierelated assumption. Such rare potential pathogenic PRNP mutations could occur in countries at present considered to be free of BSE, such as Australia and New Zealand. So it is important to maintain strict surveillance for BSE in cattle, with rigorous enforcement of the ruminant feed ban (many countries still feed ruminant proteins to pigs). Removal of specified risk material, such as brain and spinal cord, from cattle at slaughter prevents infected material from entering the human food chain. Routine genetic screening of cattle for PRNP mutations, which is now available, could provide additional data on the risk to the public. Because the point mutation identified in the Alabama animals is identical to that responsible for the commonest type of familial (genetic) CJD in humans, it is possible that the resulting infective prion protein might cross the bovine-human species barrier more easily. Patients with vCJD continue to be identified. The fact that this is happening less often should not lead to relaxation of the controls necessary to prevent future outbreaks. Malcolm A. Ferguson-Smith Cambridge University Department of Veterinary Medicine, Madingley Road, Cambridge CB3 0ES, UK e-mail: maf12@cam.ac.uk Jürgen A. Richt College of Veterinary Medicine, Kansas State University, K224B Mosier Hall, Manhattan, Kansas 66506-5601, USA NATURE|Vol 457|26 February 2009
http://www.nature.com/nature/journal/v457/n7233/full/4571079b.html
SEE FULL TEXT OF ALL THIS HERE ;
2009 UPDATE ON ALABAMA AND TEXAS MAD COWS 2005 and 2006
http://bse-atypical.blogspot.com/2006/08/bse-atypical-texas-and-alabama-update.html
Friday, December 23, 2011
Oral Transmission of L-type Bovine Spongiform Encephalopathy in Primate Model
Volume 18, Number 1—January 2012 Dispatch
http://transmissiblespongiformencephalopathy.blogspot.com/2011/12/oral-transmission-of-l-type-bovine.html
P.9.21 Molecular characterization of BSE in Canada
Jianmin Yang1, Sandor Dudas2, Catherine Graham2, Markus Czub3, Tim McAllister1, Stefanie Czub1 1Agriculture and Agri-Food Canada Research Centre, Canada; 2National and OIE BSE Reference Laboratory, Canada; 3University of Calgary, Canada
Background: Three BSE types (classical and two atypical) have been identified on the basis of molecular characteristics of the misfolded protein associated with the disease. To date, each of these three types have been detected in Canadian cattle.
Objectives: This study was conducted to further characterize the 16 Canadian BSE cases based on the biochemical properties of there associated PrPres.
Methods: Immuno-reactivity, molecular weight, glycoform profiles and relative proteinase K sensitivity of the PrPres from each of the 16 confirmed Canadian BSE cases was determined using modified Western blot analysis. Results: Fourteen of the 16 Canadian BSE cases were C type, 1 was H type and 1 was L type. The Canadian H and L-type BSE cases exhibited size shifts and changes in glycosylation similar to other atypical BSE cases. PK digestion under mild and stringent conditions revealed a reduced protease resistance of the atypical cases compared to the C-type cases. N terminal- specific antibodies bound to PrPres from H type but not from C or L type. The C-terminal-specific antibodies resulted in a shift in the glycoform profile and detected a fourth band in the Canadian H-type BSE.
Discussion: The C, L and H type BSE cases in Canada exhibit molecular characteristics similar to those described for classical and atypical BSE cases from Europe and Japan. This supports the theory that the importation of BSE contaminated feedstuff is the source of C-type BSE in Canada. *** It also suggests a similar cause or source for atypical BSE in these countries.
http://www.prion2009.com/sites/default/files/Prion2009_Book_of_Abstracts.pdf
October 2009 O.11.3 Infectivity in skeletal muscle of BASE-infected cattle
Silvia Suardi1, Chiara Vimercati1, Fabio Moda1, Ruggerone Margherita1, Ilaria Campagnani1, Guerino Lombardi2, Daniela Gelmetti2, Martin H. Groschup3, Anne Buschmann3, Cristina Casalone4, Maria Caramelli4, Salvatore Monaco5, Gianluigi Zanusso5, Fabrizio Tagliavini1 1Carlo Besta" Neurological Institute,Italy; 2IZS Brescia, Italy; 33FLI Insel Riems, D, Germany; 4CEA-IZS Torino, Italy; 5University of Verona, Italy
Background: BASE is an atypical form of bovine spongiform encephalopathy caused by a prion strain distinct from that of BSE. Upon experimental transmission to cattle, BASE induces a previously unrecognized disease phenotype marked by mental dullness and progressive atrophy of hind limb musculature. Whether affected muscles contain infectivity is unknown. This is a critical issue since the BASE strain is readily transmissible to a variety of hosts including primates, suggesting that humans may be susceptible.
Objectives: To investigate the distribution of infectivity in peripheral tissues of cattle experimentally infected with BASE. Methods: Groups of Tg mice expressing bovine PrP (Tgbov XV, n= 7-15/group) were inoculated both i.c. and i.p. with 10% homogenates of a variety of tissues including brain, spleen, cervical lymph node, kidney and skeletal muscle (m. longissimus dorsi) from cattle intracerebrally infected with BASE. No PrPres was detectable in the peripheral tissues used for inoculation either by immunohistochemistry or Western blot.
Results: Mice inoculated with BASE-brain homogenates showed clinical signs of disease with incubation and survival times of 175±15 and 207±12 days. Five out of seven mice challenged with skeletal muscle developed a similar neurological disorder, with incubation and survival times of 380±11 and 410±12 days. At present (700 days after inoculation) mice challenged with the other peripheral tissues are still healthy. The neuropathological phenotype and PrPres type of the affected mice inoculated either with brain or muscle were indistinguishable and matched those of Tgbov XV mice infected with natural BASE.
Discussion: Our data indicate that the skeletal muscle of cattle experimentally infected with BASE contains significant amount of infectivity, at variance with BSE-affected cattle, raising the issue of intraspecies transmission and the potential risk for humans. Experiments are in progress to assess the presence of infectivity in skeletal muscles of natural BASE.
http://www.prion2009.com/sites/default/files/Prion2009_Book_of_Abstracts.pdf
Saturday, July 23, 2011
CATTLE HEADS WITH TONSILS, BEEF TONGUES, SPINAL CORD, SPECIFIED RISK MATERIALS (SRM's) AND PRIONS, AKA MAD COW DISEASE
http://transmissiblespongiformencephalopathy.blogspot.com/2011/07/cattle-heads-with-tonsils-beef-tongues.html
Saturday, November 6, 2010
TAFS1 Position Paper on Position Paper on Relaxation of the Feed Ban in the EU
Berne, 2010 TAFS INTERNATIONAL FORUM FOR TRANSMISSIBLE ANIMAL DISEASES AND FOOD SAFETY a non-profit Swiss Foundation
http://madcowfeed.blogspot.com/2010/11/tafs1-position-paper-on-position-paper.html
Archive Number 20101206.4364 Published Date 06-DEC-2010 Subject PRO/AH/EDR>
Prion disease update 2010 (11) PRION DISEASE UPDATE 2010 (11)
http://www.promedmail.org/direct.php?id=20101206.4364
Sunday, February 5, 2012
February 2012 Update on Feed Enforcement Activities to Limit the Spread of BSE
http://transmissiblespongiformencephalopathy.blogspot.com/2012/02/february-2012-update-on-feed.html
Wednesday, March 7, 2012
Case-control study of cases of bovine spongiform encephalopathy born after July 31, 1996 (BARB cases) in Great Britain Veterinary Record doi:10.1136/vr.100097
http://madcowfeed.blogspot.com/2012/03/case-control-study-of-cases-of-bovine.html
Wednesday, March 7, 2012
The epidemiology of bovine spongiform encephalopathy in the Republic of Ireland before and after the reinforced feed ban
http://madcowfeed.blogspot.com/2012/03/epidemiology-of-bovine-spongiform.html
Thursday, February 23, 2012
EIGHT FORMER SECRETARIES OF AGRICULTURE SPEAKING AT USDA'S 2012 AGRICULTURE OUTLOOK FORUM INDUCTED INTO USA MAD COW HALL OF SHAME
http://madcowusda.blogspot.com/2012/02/eight-former-secretaries-of-agriculture.html
Tuesday, February 14, 2012
White House budget proposes cuts to ag programs including TSE PRION disease aka mad cow type disease
http://transmissiblespongiformencephalopathy.blogspot.com/2012/02/white-house-budget-proposes-cuts-to-ag.html
Thursday, February 16, 2012
Bovine Spongiform Encephalopathy BSE
31 USA SENATORS ASK PRESIDENT OBAMA TO HELP SPREAD MAD COW DISEASE 2012
http://transmissiblespongiformencephalopathy.blogspot.com/2012/02/bovine-spongiform-encephalopathy-bse-31.html
> > > Ackerman says downed cattle are 50 times more likely to have mad cow disease (also known as Bovine Spongiform Encephalopathy, or BSE) than ambulatory cattle that are suspected of having BSE. Of the 20 confirmed cases of mad cow disease in North America since 1993, at least 16 have involved downer cattle, he said. < < <
don’t forget the children...
PLEASE be aware, for 4 years, the USDA fed our children all across the Nation (including TEXAS) dead stock downer cows, the most high risk cattle for BSE aka mad cow disease and other dangerous pathogens.
who will watch our children for CJD for the next 5+ decades ???
WAS your child exposed to mad cow disease via the NSLP ???
SCHOOL LUNCH PROGRAM FROM DOWNER CATTLE UPDATE
http://downercattle.blogspot.com/2009/05/who-will-watch-children.html
http://downercattle.blogspot.com/
DID YOUR CHILD CONSUME SOME OF THESE DEAD STOCK DOWNER COWS, THE MOST HIGH RISK FOR MAD COW DISEASE ???
you can check and see here ;
http://www.fns.usda.gov/fns/safety/pdf/Hallmark-Westland_byState.pdf
Wednesday, March 14, 2012
PINK SLIME, MRM’s, BSE AKA MAD COW DISEASE, AND THE USDA NSLP
http://madcowusda.blogspot.com/2012/03/pink-slime-mrms-bse-aka-mad-cow-disease.html
Saturday, March 5, 2011
MAD COW ATYPICAL CJD PRION TSE CASES WITH CLASSIFICATIONS PENDING ON THE RISE IN NORTH AMERICA
http://transmissiblespongiformencephalopathy.blogspot.com/2011/03/mad-cow-atypical-cjd-prion-tse-cases.html
Sunday, February 12, 2012
National Prion Disease Pathology Surveillance Center Cases Examined1 (August 19, 2011) including Texas
http://transmissiblespongiformencephalopathy.blogspot.com/2012/02/national-prion-disease-pathology.html
Thursday, March 01, 2012
Variant Creutzfeldt-Jakob disease Fact sheet N°180 Revised February 2012 W.H.O.
http://vcjd.blogspot.com/2012/03/variant-creutzfeldt-jakob-disease-fact.html
Friday, March 09, 2012
Experimental H-type and L-type bovine spongiform encephalopathy in cattle: observation of two clinical syndromes and diagnostic challenges
Research article
http://bse-atypical.blogspot.com/2012/03/experimental-h-type-and-l-type-bovine.html
Friday, December 23, 2011
Oral Transmission of L-type Bovine Spongiform Encephalopathy in Primate Model
Volume 18, Number 1—January 2012 Dispatch
http://transmissiblespongiformencephalopathy.blogspot.com/2011/12/oral-transmission-of-l-type-bovine.html
Thursday, March 15, 2012
Another cow enters food supply without being tested for BSE aka mad cow disease UK 15 March 2012
http://transmissiblespongiformencephalopathy.blogspot.com/2012/03/another-cow-enters-food-supply-without.html
Sunday, March 11, 2012
APHIS Proposes New Bovine Spongiform Encephalopathy Import Regulations in Line with International Animal Health Standards Proposal Aims to Ensure Health of the U.S. Beef Herd, Assist in Negotiations
while at the same time, reducing safety for humans via BSE aka mad cow type disease in North America and abroad via the OIE USDA trading of all strains of TSE prion disease via the BSE MRR policy $$$
http://transmissiblespongiformencephalopathy.blogspot.com/2012/03/aphis-proposes-new-bovine-spongiform.html
THANKS TO THE USDA AND THE OIE ET AL, they expose you and your children to mad cow type disease, because they refuse to go by their own science. see their junk science and policy there from ;
Research Project: STUDY OF ATYPICAL BSE Location: Virus and Prion Research Unit
Project Number: 3625-32000-086-05 Project Type: Specific Cooperative Agreement
Start Date: Sep 15, 2004 End Date: Sep 14, 2009
Objective: The objective of this cooperative research project with Dr. Maria Caramelli from the Italian BSE Reference Laboratory in Turin, Italy, is to conduct comparative studies with the U.S. bovine spongiform encephalopathy (BSE) isolate and the atypical BSE isolates identified in Italy. The studies will cover the following areas: 1. Evaluation of present diagnostics tools used in the U.S. for the detection of atypical BSE cases. 2. Molecular comparison of the U.S. BSE isolate and other typical BSE isolates with atypical BSE cases. 3. Studies on transmissibility and tissue distribution of atypical BSE isolates in cattle and other species.
Approach: This project will be done as a Specific Cooperative Agreement with the Italian BSE Reference Laboratory, Istituto Zooprofilattico Sperimentale del Piemonte, in Turin, Italy. It is essential for the U.S. BSE surveillance program to analyze the effectiveness of the U.S diagnostic tools for detection of atypical cases of BSE. Molecular comparisons of the U.S. BSE isolate with atypical BSE isolates will provide further characterization of the U.S. BSE isolate. Transmission studies are already underway using brain homogenates from atypical BSE cases into mice, cattle and sheep. It will be critical to see whether the atypical BSE isolates behave similarly to typical BSE isolates in terms of transmissibility and disease pathogenesis. If transmission occurs, tissue distribution comparisons will be made between cattle infected with the atypical BSE isolate and the U.S. BSE isolate. Differences in tissue distribution could require new regulations regarding specific risk material (SRM) removal.
http://www.ars.usda.gov/research/projects/projects.htm?accn_no=408490
3.Progress Report This report documents research conducted under a Specific Cooperative Agreement between ARS and the IST ZOOPROFIL SPERIMENT PIEMONTE. Additional details for the research can be found in the report for the parent project 3625-32000-086-00D, TRANSMISSION, DIFFERENTIATION, AND PATHOBIOLOGY OF TRANSMISSIBLE SPONGIFORM ENCEPHALOPATHIES
The aim of the cooperative research project was to: 1. Evaluate present diagnostic tools used in the U.S. for the detection of atypical bovine spongiform encephalopathy (BSE) cases. 2. Perform molecular comparison of the U.S. BSE isolate and other typical BSE isolates with atypical BSE cases. 3. Support studies on transmissibility and tissue distribution of atypical BSE isolates in cattle and other species. To complete objectives 1 and 2 (i.e., to compare Italian and U.S. BSE confirmatory protocols for detection of classical (C-) and atypical (H- and L-type) BSE cases), samples of Italian C-BSE and Italian L-type BSE (BASE), both frozen and formalin fixed, were sent to USDA laboratories in Ames, Iowa, to undergo Western blot (WB) and immunohistochemistry (IHC) comparison studies for PrPSc detection according to U.S. and Italian methods. A Western blot expert from the cooperating Italian lab assisted ARS scientists in performing the protocols from each laboratory in parallel. A comparative IHC study between U.S. and Italian BSE confirmatory protocols was also performed when the collaborator sent a scientist to Ames to assist in performing the Italian IHC protocol on BSE samples chosen for the study. Results obtained showed the Italian and U.S. IHC procedures were alike in PrPSc detection regarding its tissue distribution, deposition pattern and intensity of staining on all the C-, L- and H-type BSE cases considered. In addition, the U.S. protocol evidenced the characteristic presence of plaques in the frontal cortex of the Italian BASE case similar to the Italian protocol. Data from studies on objectives 1 & 2 has been presented at several international meetings in 2008 and 2009, and has been finalized into manuscript form for publication in a peer-reviewed journal (Journal of Veterinary Diagnostic Investigation). In support of objective 3, the cooperators completed and published their transmissibility and tissue distribution work on BASE cases in a peer-reviewed journal in 2008 (PLoS Pathogens Volume 4, page e1000075). They reported that in all experimentally infected atypical BSE animals, no PrPSc was detected in peripheral tissues either by standard Western blot analysis or following phosphotungstic acid precipitation. Peripheral issues examined included cervical and mesenteric lymph nodes, spleen, thymus, liver, lung, peripheral nerves, and forelimb and hind limb muscles. These findings support the conclusion there is no scientific evidence to expand the list of tissues included in the Specified Risk Material ban based on atypical BSE research data, thus confirming other studies indicating the pathogenesis of BSE in cattle is fundamentally different from that in sheep and mice, due to an exclusive intraneuronal spread of infectivity from the gut to the central nervous system. Methods used for monitoring included email, site visits, and periodic written reports.
http://www.ars.usda.gov/research/projects/projects.htm?ACCN_NO=408490&showpars=true&fy=2010
THE USDA AND THE OIE are industry friendly groups, tied to the hips for one reason, TRADE $$$
THERE is nothing science based about policy making for the TSE prion disease with neither one of these two groups i.e. OIE and the USDA.
IN MY OPINION, both organizations should be shut down for good, with an International Tribunal for their gross negligence with the TSE prion disease, thus, exposing humans and animals around the globe.
HOW many more will die ?
Neither the OIE or the USDA care about a slow incubating disease (as long as 50 years in some cases), that is 100% fatal, due to the fact that since the long incubation period, there is no way to trace back source.
I assure you, both the OIE and the USDA plan to keep it that way. ...
DEEP THROAT TO TSS 2000-2001 (take these old snips of emails with how ever many grains of salt you wish. ...tss)
The most frightening thing I have read all day is the report of Gambetti's finding of a new strain of sporadic cjd in young people...Dear God, what in the name of all that is holy is that!!! If the US has different strains of scrapie.....why????than the UK...then would the same mechanisms that make different strains of scrapie here make different strains of BSE...if the patterns are different in sheep and mice for scrapie.....could not the BSE be different in the cattle, in the mink, in the humans.......I really think the slides or tissues and everything from these young people with the new strain of sporadic cjd should be put up to be analyzed by many, many experts in cjd........bse.....scrapie Scrape the damn slide and put it into mice.....wait.....chop up the mouse brain and and spinal cord........put into some more mice.....dammit amplify the thing and start the damned research.....This is NOT rocket science...we need to use what we know and get off our butts and move....the whining about how long everything takes.....well it takes a whole lot longer if you whine for a year and then start the research!!! Not sure where I read this but it was a recent press release or something like that: I thought I would fall out of my chair when I read about how there was no worry about infectivity from a histopath slide or tissues because they are preserved in formic acid, or formalin or formaldehyde.....for God's sake........ Ask any pathologist in the UK what the brain tissues in the formalin looks like after a year.......it is a big fat sponge...the agent continues to eat the brain ......you can't make slides anymore because the agent has never stopped........and the old slides that are stained with Hemolysin and Eosin......they get holier and holier and degenerate and continue...what you looked at 6 months ago is not there........Gambetti better be photographing every damned thing he is looking at.....
Okay, you need to know. You don't need to pass it on as nothing will come of it and there is not a damned thing anyone can do about it. Don't even hint at it as it will be denied and laughed at.......... USDA is gonna do as little as possible until there is actually a human case in the USA of the nvcjd........if you want to move this thing along and shake the earth....then we gotta get the victims families to make sure whoever is doing the autopsy is credible, trustworthy, and a saint with the courage of Joan of Arc........I am not kidding!!!! so, unless we get a human death from EXACTLY the same form with EXACTLY the same histopath lesions as seen in the UK nvcjd........forget any action........it is ALL gonna be sporadic!!!
And, if there is a case.......there is gonna be every effort to link it to international travel, international food, etc. etc. etc. etc. etc. They will go so far as to find out if a sex partner had ever traveled to the UK/europe, etc. etc. .... It is gonna be a long, lonely, dangerous twisted journey to the truth. They have all the cards, all the money, and are willing to threaten and carry out those threats....and this may be their biggest downfall...
Thanks as always for your help. (Recently had a very startling revelation from a rather senior person in government here..........knocked me out of my chair........you must keep pushing. If I was a power person....I would be demanding that there be a least a million bovine tested as soon as possible and agressively seeking this disease. The big players are coming out of the woodwork as there is money to be made!!! In short: "FIRE AT WILL"!!! for the very dumb....who's "will"! "Will be the burden to bare if there is any coverup!"
again it was said years ago and it should be taken seriously....BSE will NEVER be found in the US! As for the BSE conference call...I think you did a great service to freedom of information and making some people feign integrity...I find it scary to see that most of the "experts" are employed by the federal government or are supported on the "teat" of federal funds. A scary picture! I hope there is a confidential panel organized by the new government to really investigate this thing.
You need to watch your back........but keep picking at them.......like a buzzard to the bone...you just may get to the truth!!! (You probably have more support than you know. Too many people are afraid to show you or let anyone else know. I have heard a few things myself... you ask the questions that everyone else is too afraid to ask.)
END...TSS
IN CONFIDENCE
Perceptions of unconventional slow virus in the USA
GAH WELLS
Report of a visit to the U.S.A. April-May 1989
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 fantical incident to be avoided in the USA AT ALL COSTS.
http://web.archive.org/web/20060307063531/http://www.bseinquiry.gov.uk/files/mb/m11b/tab01.pdf
and they meant it !
TSS
Published online 2012 February 21. doi: 10.1371/journal.pone.0031449
PMCID: PMC3283643
Copyright Suardi et al. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.
Infectivity in Skeletal Muscle of Cattle with Atypical Bovine Spongiform Encephalopathy
Silvia Suardi,#1 Chiara Vimercati,#1 Cristina Casalone,#2 Daniela Gelmetti,3 Cristiano Corona,2 Barbara Iulini,2 Maria Mazza,2 Guerino Lombardi,3 Fabio Moda,1 Margherita Ruggerone,1 Ilaria Campagnani,1 Elena Piccoli,1 Marcella Catania,1 Martin H. Groschup,4 Anne Balkema-Buschmann,4 Maria Caramelli,2 Salvatore Monaco,5 Gianluigi Zanusso,5 and Fabrizio Tagliavini1*
1Instituto Di Ricoveroe Cura a Carattere Scientifico (IRCCS), Foundation “Carlo Besta” Neurological Institute, Milano, Italy
2Istituto Zooprofilattico Sperimentale del Piemonte, Liguria e Valle d'Aosta, Torino, Italy
3Istituto Zooprofilattico Sperimentale della Lombardia ed Emilia Romagna, Brescia, Italy
4Friedrich-Loeffler-Institut, Greifswald, Insel Riems, Germany
5Policlinico G.B. Rossi, University of Verona, Verona, Italy
Jason Bartz, Editor
Creighton University, United States of America
#Contributed equally.
* E-mail: ftagliavini@istituto-besta.it
Conceived and designed the experiments: SS CV C. Casalone DG C. Corona GL SM M. Caramelli GZ FT. Performed the experiments: SS CV DG C. Corona BI MM FM MR IC EP M. Catania. Analyzed the data: SS CV C. Casalone DG C. Corona GL SM MHG AB M. Caramelli GZ FT. Contributed reagents/materials/analysis tools: SS CV C. Casalone DG C. Corona GL SM MHG AB M. Caramelli GZ FT. Wrote the paper: SS CV C. Casalone DG C. Corona GL SM MHG AB M.Caramelli GZ FT.
Received December 13, 2011; Accepted January 8, 2012.
Abstract
The amyloidotic form of bovine spongiform encephalopathy (BSE) termed BASE is caused by a prion strain whose biological properties differ from those of typical BSE, resulting in a clinically and pathologically distinct phenotype. Whether peripheral tissues of BASE-affected cattle contain infectivity is unknown. This is a critical issue since the BASE prion is readily transmissible to a variety of hosts including primates, suggesting that humans may be susceptible. We carried out bioassays in transgenic mice overexpressing bovine PrP (Tgbov XV) and found infectivity in a variety of skeletal muscles from cattle with natural and experimental BASE. Noteworthy, all BASE muscles used for inoculation transmitted disease, although the attack rate differed between experimental and natural cases (~70% versus ~10%, respectively). This difference was likely related to different prion titers, possibly due to different stages of disease in the two conditions, i.e. terminal stage in experimental BASE and pre-symptomatic stage in natural BASE. The neuropathological phenotype and PrPres type were consistent in all affected mice and matched those of Tgbov XV mice infected with brain homogenate from natural BASE. The immunohistochemical analysis of skeletal muscles from cattle with natural and experimental BASE showed the presence of abnormal prion protein deposits within muscle fibers. Conversely, Tgbov XV mice challenged with lymphoid tissue and kidney from natural and experimental BASE did not develop disease. The novel information on the neuromuscular tropism of the BASE strain, efficiently overcoming species barriers, underlines the relevance of maintaining an active surveillance.
snip...
Introduction
In 2004 an atypical form of bovine spongiform encephalopathy (BSE) termed BASE or BSE-L was identified in Italy through active surveillance [1] and subsequently recognized in different European countries, North America, Canada and Japan [1]–[6]. BASE affects relatively old cattle (all cases were older than 9 years) and differs from BSE with regard to the neuropathological phenotype, the biochemical profile of the disease-associated prion protein (PrPSc) and the biological properties of the agent strain [1], [3], [7]. The neuropathological hallmark of BASE is the presence of PrP amyloid plaques and the preferential involvement of olfactory areas, hippocampus and thalamus with a relatively low involvement of the brainstem, as opposed to classical BSE. The molecular signature of BASE is a PrPSc type distinguished by a protease-resistant core (PrPres) of lower molecular mass than BSE-PrPSc, with predominance of the monoglycosylated protein band by western immunoblot.
Intra-species transmission revealed that the clinical picture of BASE differs substantially from that of BSE, being characterized by mental dullness and amyotrophy rather than hyper-reactivity and aggressiveness [8], [9]. As a consequence, the recognition of BASE in vivo can be difficult and may represent a major challenge for passive surveillance. Transmission studies to transgenic mice overexpressing bovine PrP (Tgbov mice) showed that the BASE strain is more aggressive than the BSE strain [10]. Furthermore, Tg mice overexpressing human PrP as well as non-human primates are more susceptible to infection with BASE than with BSE [11]–[14]. Overall these data raise concern about the potential risk of transmission of BASE to humans and it is urgent to determine the presence and distribution of infectivity in peripheral tissues of BASE-affected cattle. To investigate this issue, we inoculated Tgbov mice with different peripheral tissues from experimentally and naturally BASE-affected cattle and found that various skeletal muscles contained infectivity and PrP-immunoreactive deposits within individual fibers.
snip...
Discussion
This is the first report on the occurrence and distribution of infectivity in peripheral tissues of BASE-affected cattle. We found that different skeletal muscles (i.e., M. longissimus dorsi, M. intercostalis and M. gluteus) from experimental and natural BASE carried infectivity, whereas spleen, cervical lymph node and kidney did not, as highly susceptible Tgbov XV mice did not develop disease up to 850 days after challenge. Noteworthy, all BASE muscles used for inoculation transmitted disease, although the attack rate differed between experimental and natural cases. This difference is likely related to different prion titers in the two conditions, possibly due to different stages of disease. In fact, the experimentally infected cattle was sacrificed at the terminal stage when clinical signs were severe [8], while the cattle with natural BASE was identified through active surveillance at a pre-symptomatic stage.
Infectivity in skeletal muscles has been detected in various natural and experimental prion diseases, including sheep affected by classical and Nor/98 atypical scrapie [17], deer with chronic wasting disease [18] and rodent models of scrapie [19]. A large pathogenesis study on BSE-infected cattle in the UK failed to detect infectivity in muscle tissue at any time during the course of the disease by inoculation of inbred mice [20]. However, in a subsequent study with Tgbov XV mice, M. semitendinosus from a field case of clinically symptomatic BSE transmitted disease to one out of ten inoculated rodents. This low amount of infectivity was tentatively related to the terminal nerve fibres [21].
A major clinical feature of cattle experimentally infected with BASE is amyotrophy, as a result of motor neuron disease [8]. Although we were unable to detect PrPSc in the peripheral nerves of infected cattle and follow the kinetics of PrP spreading through the neural pathway, a study of intra-species transmission of a Japanese case of BASE showed that PrPres was first detectable by immunoblot in the nerve roots and subsequently in the peripheral nerves [22]. PrPres deposition in skeletal muscles has been found in a variety of prion diseases, including experimental scrapie in hamsters [23], natural scrapie in sheep [24], and mouse and primate models of BSE and CJD [25]–[27]. These reports agree that PrPres in muscle tissue is associated with terminal nerve endings.
In our study, immunohistochemistry showed the presence of amorphous or granular, small PrP deposits in different skeletal muscles from both experimental and natural BASE-affected cattle. PrP aggregates were found in isolated muscle fibers with a scattered distribution which was at variance with the topology of PrPres reported in the previous studies [23], [24], [26], [27]. Noteworthy, identical results were obtained using two different fixatives and immunostaining protocols. Since PrPC is expressed in bovine muscles and skeletal muscles are intrinsically capable of propagating prions [19], [28], we argue that PrPSc deposition in BASE muscles might be the result of a primary PrP replication through a neural-independent pathway. This possibility has been previously considered in a patient affected by sporadic CJD and inclusion body myositis where PrPC to PrPSc conversion occurred in skeletal muscle [29].
The limited number and irregular distribution of PrP positive fibers within a muscle sample accounts for the absence of a detectable PrPres signal on Western blot. The inhomogeneous distribution of PrPres and infectivity among different muscles of the same animal and within the same muscle has been previously observed in mice and in primates infected with different prion strains. These findings have been tentatively related to biochemical differences in skeletal muscles of different body regions and/or number of nerve endings [19], [25], [26].
In the present study, Tgbov XV mice challenged with BASE muscles reproduced the monoglycosylated-dominant PrPres type of BASE as well as a histopathological lesion profile and pattern of PrPres deposition that matched those observed in mice challenged with BASE brain. These findings indicate that, in Tgbov XV mice, muscle and brain tissues maintain the same biological properties of the BASE strain.
Although serial transmission studies in Tgbov XV mice showed that the BASE prion is capable to replicate in the spleen converting, in part, into a BSE-like strain (personal observation), we did not detect infectivity in lymph node and spleen from cattle with experimental and natural BASE. This observation opposes the possibility that the BASE agent might re-circulate, potentially as BSE-like strain, in different hosts. Kidney is an edible organ and infectivity found in urine is kidney-associated [30]. Lack of transmission from kidney of natural and experimental BASE is an important issue, since infectivity in kidney has been demonstrated by bioassay in human prion diseases [31], and PrPSc has been observed in kidney of scrapie infected sheep and CWD affected deer [32]–[34].
The present data offer novel information on the tropism of the BASE agent and highlight relevant public health issues. While the transmission barrier for classical BSE is high in most species, BASE prions are readily transmissible to a variety of mammals including non-human primates [11]–[13], [35]. Accordingly, the possibility of spreading of BASE prions through skeletal muscle to other species should be taken into account and evaluated in risk analysis studies.
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3283643/?tool=pubmed
Saturday, June 25, 2011
Transmissibility of BSE-L and Cattle-Adapted TME Prion Strain to Cynomolgus Macaque
"BSE-L in North America may have existed for decades"
http://transmissiblespongiformencephalopathy.blogspot.com/2011/06/transmissibility-of-bse-l-and-cattle.html
Over the next 8-10 weeks, approximately 40% of all the adult mink on the farm died from TME.
snip...
The rancher was a ''dead stock'' feeder using mostly (>95%) downer or dead dairy cattle...
http://web.archive.org/web/20030516051623/http://www.bseinquiry.gov.uk/files/mb/m09/tab05.pdf
Monday, October 10, 2011
EFSA Journal 2011 The European Response to BSE: A Success Story
snip...
EFSA and the European Centre for Disease Prevention and Control (ECDC) recently delivered a scientific opinion on any possible epidemiological or molecular association between TSEs in animals and humans (EFSA Panel on Biological Hazards (BIOHAZ) and ECDC, 2011). This opinion confirmed Classical BSE prions as the only TSE agents demonstrated to be zoonotic so far but the possibility that a small proportion of human cases so far classified as "sporadic" CJD are of zoonotic origin could not be excluded. Moreover, transmission experiments to non-human primates suggest that some TSE agents in addition to Classical BSE prions in cattle (namely L-type Atypical BSE, Classical BSE in sheep, transmissible mink encephalopathy (TME) and chronic wasting disease (CWD) agents) might have zoonotic potential.
snip...
http://www.efsa.europa.eu/en/efsajournal/pub/e991.htm?emt=1
http://www.efsa.europa.eu/en/efsajournal/doc/e991.pdf
see follow-up here about North America BSE Mad Cow TSE prion risk factors, and the ever emerging strains of Transmissible Spongiform Encephalopathy in many species here in the USA, including humans ;
http://transmissiblespongiformencephalopathy.blogspot.com/2011/10/efsa-journal-2011-european-response-to.html
Thursday, August 12, 2010
Seven main threats for the future linked to prions
First threat
The TSE road map defining the evolution of European policy for protection against prion diseases is based on a certain numbers of hypotheses some of which may turn out to be erroneous. In particular, a form of BSE (called atypical Bovine Spongiform Encephalopathy), recently identified by systematic testing in aged cattle without clinical signs, may be the origin of classical BSE and thus potentially constitute a reservoir, which may be impossible to eradicate if a sporadic origin is confirmed.
***Also, a link is suspected between atypical BSE and some apparently sporadic cases of Creutzfeldt-Jakob disease in humans. These atypical BSE cases constitute an unforeseen first threat that could sharply modify the European approach to prion diseases.
Second threat
snip...
http://www.neuroprion.org/en/np-neuroprion.html
Rural and Regional Affairs and Transport References Committee
The possible impacts and consequences for public health, trade and agriculture of the Government's decision to relax import restrictions on beef Final report June 2010
2.65 At its hearing on 14 May 2010, the committee heard evidence from Dr Alan Fahey who has recently submitted a thesis on the clinical neuropsychiatric, epidemiological and diagnostic features of Creutzfeldt-Jakob disease.48 Dr Fahey told the committee of his concerns regarding the lengthy incubation period for transmissible spongiform encephalopathies, the inadequacy of current tests and the limited nature of our current understanding of this group of diseases.49
2.66 Dr Fahey also told the committee that in the last two years a link has been established between forms of atypical CJD and atypical BSE. Dr Fahey said that: They now believe that those atypical BSEs overseas are in fact causing sporadic Creutzfeldt-Jakob disease. They were not sure if it was due to mad sheep disease or a different form. If you look in the textbooks it looks like this is just arising by itself. But in my research I have a summary of a document which states that there has never been any proof that sporadic Creutzfeldt-Jakob disease has arisen de novo-has arisen of itself. There is no proof of that. The recent research is that in fact it is due to atypical forms of mad cow disease which have been found across Europe, have been found in America and have been found in Asia. These atypical forms of mad cow disease typically have even longer incubation periods than the classical mad cow disease.50
http://www.aph.gov.au/senate/committee/rrat_ctte/mad_cows/report/report.pdf
Atypical BSE in Cattle
To date the OIE/WAHO assumes that the human and animal health standards set out in the BSE chapter for classical BSE (C-Type) applies to all forms of BSE which include the H-type and L-type atypical forms. This assumption is scientifically not completely justified and accumulating evidence suggests that this may in fact not be the case. Molecular characterization and the spatial distribution pattern of histopathologic lesions and immunohistochemistry (IHC) signals are used to identify and characterize atypical BSE. Both the L-type and H-type atypical cases display significant differences in the conformation and spatial accumulation of the disease associated prion protein (PrPSc) in brains of afflicted cattle. Transmission studies in bovine transgenic and wild type mouse models support that the atypical BSE types might be unique strains because they have different incubation times and lesion profiles when compared to C-type BSE. When L-type BSE was inoculated into ovine transgenic mice and Syrian hamster the resulting molecular fingerprint had changed, either in the first or a subsequent passage, from L-type into C-type BSE.
In addition, non-human primates are specifically susceptible for atypical BSE as demonstrated by an approximately 50% shortened incubation time for L-type BSE as compared to C-type. Considering the current scientific information available, it cannot be assumed that these different BSE types pose the same human health risks as C-type BSE or that these risks are mitigated by the same protective measures.
This study will contribute to a correct definition of specified risk material (SRM) in atypical BSE. The incumbent of this position will develop new and transfer existing, ultra-sensitive methods for the detection of atypical BSE in tissue of experimentally infected cattle.
http://www.prionetcanada.ca/detail.aspx?menu=5&dt=293380&app=93&cat1=387&tp=20&lk=no&cat2
When L-type BSE was inoculated into ovine transgenic mice and Syrian hamster the resulting molecular fingerprint had changed, either in the first or a subsequent passage, from L-type into C-type BSE. In addition, non-human primates are specifically susceptible for atypical BSE as demonstrated by an approximately 50% shortened incubation time for L-type BSE as compared to C-type. Considering the current scientific information available, it cannot be assumed that these different BSE types pose the same human health risks as C-type BSE or that these risks are mitigated by the same protective measures.
This study will contribute to a correct definition of specified risk material (SRM) in atypical BSE. The incumbent of this position will develop new and transfer existing, ultra-sensitive methods for the detection of atypical BSE in tissue of experimentally infected cattle.
http://www.prionetcanada.ca/detail.aspx?menu=5&dt=293380&app=93&cat1=387&tp=20&lk=no&cat2
P.4.23
Transmission of atypical BSE in humanized mouse models
Liuting Qing1, Wenquan Zou1, Cristina Casalone2, Martin Groschup3, Miroslaw Polak4, Maria Caramelli2, Pierluigi Gambetti1, Juergen Richt5, Qingzhong Kong1 1Case Western Reserve University, USA; 2Instituto Zooprofilattico Sperimentale, Italy; 3Friedrich-Loeffler-Institut, Germany; 4National Veterinary Research Institute, Poland; 5Kansas State University (Previously at USDA National Animal Disease Center), USA
Background: Classical BSE is a world-wide prion disease in cattle, and the classical BSE strain (BSE-C) has led to over 200 cases of clinical human infection (variant CJD). Atypical BSE cases have been discovered in three continents since 2004; they include the L-type (also named BASE), the H-type, and the first reported case of naturally occurring BSE with mutated bovine PRNP (termed BSE-M). The public health risks posed by atypical BSE were largely undefined.
Objectives: To investigate these atypical BSE types in terms of their transmissibility and phenotypes in humanized mice. Methods: Transgenic mice expressing human PrP were inoculated with several classical (C-type) and atypical (L-, H-, or Mtype) BSE isolates, and the transmission rate, incubation time, characteristics and distribution of PrPSc, symptoms, and histopathology were or will be examined and compared.
Results: Sixty percent of BASE-inoculated humanized mice became infected with minimal spongiosis and an average incubation time of 20-22 months, whereas only one of the C-type BSE-inoculated mice developed prion disease after more than 2 years. Protease-resistant PrPSc in BASE-infected humanized Tg mouse brains was biochemically different from bovine BASE or sCJD. PrPSc was also detected in the spleen of 22% of BASE-infected humanized mice, but not in those infected with sCJD. Secondary transmission of BASE in the humanized mice led to a small reduction in incubation time.*** The atypical BSE-H strain is also transmissible with distinct phenotypes in the humanized mice, but no BSE-M transmission has been observed so far.
Discussion: Our results demonstrate that BASE is more virulent than classical BSE, has a lymphotropic phenotype, and displays a modest transmission barrier in our humanized mice. BSE-H is also transmissible in our humanized Tg mice. The possibility of more than two atypical BSE strains will be discussed.
Supported by NINDS NS052319, NIA AG14359, and NIH AI 77774.
http://www.prion2009.com/sites/default/files/Prion2009_Book_of_Abstracts.pdf
P26 TRANSMISSION OF ATYPICAL BOVINE SPONGIFORM ENCEPHALOPATHY (BSE) IN HUMANIZED MOUSE MODELS
Liuting Qing1, Fusong Chen1, Michael Payne1, Wenquan Zou1, Cristina Casalone2, Martin Groschup3, Miroslaw Polak4, Maria Caramelli2, Pierluigi Gambetti1, Juergen Richt5*, and Qingzhong Kong1 1Department of Pathology, Case Western Reserve University, Cleveland, OH 44106, USA; 2CEA, Istituto Zooprofilattico Sperimentale, Italy; 3Friedrich-Loeffler-Institut, Germany; 4National Veterinary Research Institute, Poland; 5Kansas State University, Diagnostic Medicine/Pathobiology Department, Manhattan, KS 66506, USA. *Previous address: USDA National Animal Disease Center, Ames, IA 50010, USA
Classical BSE is a world-wide prion disease in cattle, and the classical BSE strain (BSE-C) has led to over 200 cases of clinical human infection (variant CJD). Two atypical BSE strains, BSE-L (also named BASE) and BSE-H, have been discovered in three continents since 2004. The first case of naturally occurring BSE with mutated bovine PrP gene (termed BSE-M) was also found in 2006 in the USA. The transmissibility and phenotypes of these atypical BSE strains/isolates in humans were unknown. We have inoculated humanized transgenic mice with classical and atypical BSE strains (BSE-C, BSE-L, BSE-H) and the BSE-M isolate. We have found that the atypical BSE-L strain is much more virulent than the classical BSE-C.*** The atypical BSE-H strain is also transmissible in the humanized transgenic mice with distinct phenotype, but no transmission has been observed for the BSE-M isolate so far.
III International Symposium on THE NEW PRION BIOLOGY: BASIC SCIENCE, DIAGNOSIS AND THERAPY 2 - 4 APRIL 2009, VENEZIA (ITALY)
http://www.istitutoveneto.it/prion_09/Abstracts_09.pdf
I ask Professor Kong ;
Thursday, December 04, 2008 3:37 PM Subject: RE: re--Chronic Wating Disease (CWD) and Bovine Spongiform Encephalopathies (BSE): Public Health Risk Assessment
''IS the h-BSE more virulent than typical BSE as well, or the same as cBSE, or less virulent than cBSE? just curious.....''
Professor Kong reply ;
.....snip
''As to the H-BSE, we do not have sufficient data to say one way or another, but we have found that H-BSE can infect humans. I hope we could publish these data once the study is complete. Thanks for your interest.''
Best regards, Qingzhong Kong, PhD Associate Professor Department of Pathology Case Western Reserve University Cleveland, OH 44106 USA
END...TSS
Thursday, December 04, 2008 2:37 PM
"we have found that H-BSE can infect humans."
personal communication with Professor Kong. ...TSS
BSE-H is also transmissible in our humanized Tg mice.
The possibility of more than two atypical BSE strains will be discussed.
Supported by NINDS NS052319, NIA AG14359, and NIH AI 77774.
http://www.prion2009.com/sites/default/files/Prion2009_Book_of_Abstracts.pdf
http://transmissiblespongiformencephalopathy.blogspot.com/2011/06/experimental-h-type-bovine-spongiform.html
Tuesday, July 14, 2009 U.S.
Emergency Bovine Spongiform Encephalopathy Response Plan Summary and BSE Red Book
Date: February 14, 2000 at 8:56 am PST
WHERE did we go wrong $$$
http://madcowtesting.blogspot.com/2009/07/us-emergency-bovine-spongiform.html
LET'S take a closer look at this new prionpathy or prionopathy, and then let's look at the g-h-BSEalabama mad cow. This new prionopathy in humans? the genetic makeup is IDENTICAL to the g-h-BSEalabama mad cow, the only _documented_ mad cow in the world to date like this, ......wait, it get's better. this new prionpathy is killing young and old humans, with LONG DURATION from onset of symptoms to death, and the symptoms are very similar to nvCJD victims, OH, and the plaques are very similar in some cases too, bbbut, it's not related to the g-h-BSEalabama cow, WAIT NOW, it gets even better, the new human prionpathy that they claim is a genetic TSE, has no relation to any gene mutation in that family. daaa, ya think it could be related to that mad cow with the same genetic make-up ??? there were literally tons and tons of banned mad cow protein in Alabama in commerce, and none of it transmitted to cows, and the cows to humans there from ??? r i g h t $$$ ALABAMA MAD COW g-h-BSEalabama In this study, we identified a novel mutation in the bovine prion protein gene (Prnp), called E211K, of a confirmed BSE positive cow from Alabama, United States of America. This mutation is identical to the E200K pathogenic mutation found in humans with a genetic form of CJD. This finding represents the first report of a confirmed case of BSE with a potential pathogenic mutation within the bovine Prnp gene. We hypothesize that the bovine Prnp E211K mutation most likely has caused BSE in "the approximately 10-year-old cow" carrying the E221K mutation.
http://www.plospathogens.org/article/info%3Adoi%2F10.1371%2Fjournal.ppat.1000156
http://www.plospathogens.org/article/fetchObjectAttachment.action?uri=info%3Adoi%2F10.1371%2Fjournal.ppat.1000156&representation=PDF
Saturday, August 14, 2010
BSE Case Associated with Prion Protein Gene Mutation (g-h-BSEalabama) and VPSPr PRIONPATHY (see mad cow feed in COMMERCE IN ALABAMA...TSS)
http://prionpathy.blogspot.com/2010/08/bse-case-associated-with-prion-protein.html
her healthy calf also carried the mutation (J. A. Richt and S. M. Hall PLoS Pathog. 4, e1000156; 2008).
This raises the possibility that the disease could occasionally be genetic in origin. Indeed, the report of the UK BSE Inquiry in 2000 suggested that the UK epidemic had most likely originated from such a mutation and argued against the scrapierelated assumption. Such rare potential pathogenic PRNP mutations could occur in countries at present considered to be free of BSE, such as Australia and New Zealand. So it is important to maintain strict surveillance for BSE in cattle, with rigorous enforcement of the ruminant feed ban (many countries still feed ruminant proteins to pigs). Removal of specified risk material, such as brain and spinal cord, from cattle at slaughter prevents infected material from entering the human food chain. Routine genetic screening of cattle for PRNP mutations, which is now available, could provide additional data on the risk to the public. Because the point mutation identified in the Alabama animals is identical to that responsible for the commonest type of familial (genetic) CJD in humans, it is possible that the resulting infective prion protein might cross the bovine-human species barrier more easily. Patients with vCJD continue to be identified. The fact that this is happening less often should not lead to relaxation of the controls necessary to prevent future outbreaks. Malcolm A. Ferguson-Smith Cambridge University Department of Veterinary Medicine, Madingley Road, Cambridge CB3 0ES, UK e-mail: maf12@cam.ac.uk Jürgen A. Richt College of Veterinary Medicine, Kansas State University, K224B Mosier Hall, Manhattan, Kansas 66506-5601, USA NATURE|Vol 457|26 February 2009
http://www.nature.com/nature/journal/v457/n7233/full/4571079b.html
SEE FULL TEXT OF ALL THIS HERE ;
2009 UPDATE ON ALABAMA AND TEXAS MAD COWS 2005 and 2006
http://bse-atypical.blogspot.com/2006/08/bse-atypical-texas-and-alabama-update.html
Friday, December 23, 2011
Oral Transmission of L-type Bovine Spongiform Encephalopathy in Primate Model
Volume 18, Number 1—January 2012 Dispatch
http://transmissiblespongiformencephalopathy.blogspot.com/2011/12/oral-transmission-of-l-type-bovine.html
P.9.21 Molecular characterization of BSE in Canada
Jianmin Yang1, Sandor Dudas2, Catherine Graham2, Markus Czub3, Tim McAllister1, Stefanie Czub1 1Agriculture and Agri-Food Canada Research Centre, Canada; 2National and OIE BSE Reference Laboratory, Canada; 3University of Calgary, Canada
Background: Three BSE types (classical and two atypical) have been identified on the basis of molecular characteristics of the misfolded protein associated with the disease. To date, each of these three types have been detected in Canadian cattle.
Objectives: This study was conducted to further characterize the 16 Canadian BSE cases based on the biochemical properties of there associated PrPres.
Methods: Immuno-reactivity, molecular weight, glycoform profiles and relative proteinase K sensitivity of the PrPres from each of the 16 confirmed Canadian BSE cases was determined using modified Western blot analysis. Results: Fourteen of the 16 Canadian BSE cases were C type, 1 was H type and 1 was L type. The Canadian H and L-type BSE cases exhibited size shifts and changes in glycosylation similar to other atypical BSE cases. PK digestion under mild and stringent conditions revealed a reduced protease resistance of the atypical cases compared to the C-type cases. N terminal- specific antibodies bound to PrPres from H type but not from C or L type. The C-terminal-specific antibodies resulted in a shift in the glycoform profile and detected a fourth band in the Canadian H-type BSE.
Discussion: The C, L and H type BSE cases in Canada exhibit molecular characteristics similar to those described for classical and atypical BSE cases from Europe and Japan. This supports the theory that the importation of BSE contaminated feedstuff is the source of C-type BSE in Canada. *** It also suggests a similar cause or source for atypical BSE in these countries.
http://www.prion2009.com/sites/default/files/Prion2009_Book_of_Abstracts.pdf
October 2009 O.11.3 Infectivity in skeletal muscle of BASE-infected cattle
Silvia Suardi1, Chiara Vimercati1, Fabio Moda1, Ruggerone Margherita1, Ilaria Campagnani1, Guerino Lombardi2, Daniela Gelmetti2, Martin H. Groschup3, Anne Buschmann3, Cristina Casalone4, Maria Caramelli4, Salvatore Monaco5, Gianluigi Zanusso5, Fabrizio Tagliavini1 1Carlo Besta" Neurological Institute,Italy; 2IZS Brescia, Italy; 33FLI Insel Riems, D, Germany; 4CEA-IZS Torino, Italy; 5University of Verona, Italy
Background: BASE is an atypical form of bovine spongiform encephalopathy caused by a prion strain distinct from that of BSE. Upon experimental transmission to cattle, BASE induces a previously unrecognized disease phenotype marked by mental dullness and progressive atrophy of hind limb musculature. Whether affected muscles contain infectivity is unknown. This is a critical issue since the BASE strain is readily transmissible to a variety of hosts including primates, suggesting that humans may be susceptible.
Objectives: To investigate the distribution of infectivity in peripheral tissues of cattle experimentally infected with BASE. Methods: Groups of Tg mice expressing bovine PrP (Tgbov XV, n= 7-15/group) were inoculated both i.c. and i.p. with 10% homogenates of a variety of tissues including brain, spleen, cervical lymph node, kidney and skeletal muscle (m. longissimus dorsi) from cattle intracerebrally infected with BASE. No PrPres was detectable in the peripheral tissues used for inoculation either by immunohistochemistry or Western blot.
Results: Mice inoculated with BASE-brain homogenates showed clinical signs of disease with incubation and survival times of 175±15 and 207±12 days. Five out of seven mice challenged with skeletal muscle developed a similar neurological disorder, with incubation and survival times of 380±11 and 410±12 days. At present (700 days after inoculation) mice challenged with the other peripheral tissues are still healthy. The neuropathological phenotype and PrPres type of the affected mice inoculated either with brain or muscle were indistinguishable and matched those of Tgbov XV mice infected with natural BASE.
Discussion: Our data indicate that the skeletal muscle of cattle experimentally infected with BASE contains significant amount of infectivity, at variance with BSE-affected cattle, raising the issue of intraspecies transmission and the potential risk for humans. Experiments are in progress to assess the presence of infectivity in skeletal muscles of natural BASE.
http://www.prion2009.com/sites/default/files/Prion2009_Book_of_Abstracts.pdf
Saturday, July 23, 2011
CATTLE HEADS WITH TONSILS, BEEF TONGUES, SPINAL CORD, SPECIFIED RISK MATERIALS (SRM's) AND PRIONS, AKA MAD COW DISEASE
http://transmissiblespongiformencephalopathy.blogspot.com/2011/07/cattle-heads-with-tonsils-beef-tongues.html
Saturday, November 6, 2010
TAFS1 Position Paper on Position Paper on Relaxation of the Feed Ban in the EU
Berne, 2010 TAFS INTERNATIONAL FORUM FOR TRANSMISSIBLE ANIMAL DISEASES AND FOOD SAFETY a non-profit Swiss Foundation
http://madcowfeed.blogspot.com/2010/11/tafs1-position-paper-on-position-paper.html
Archive Number 20101206.4364 Published Date 06-DEC-2010 Subject PRO/AH/EDR>
Prion disease update 2010 (11) PRION DISEASE UPDATE 2010 (11)
http://www.promedmail.org/direct.php?id=20101206.4364
Sunday, February 5, 2012
February 2012 Update on Feed Enforcement Activities to Limit the Spread of BSE
http://transmissiblespongiformencephalopathy.blogspot.com/2012/02/february-2012-update-on-feed.html
Wednesday, March 7, 2012
Case-control study of cases of bovine spongiform encephalopathy born after July 31, 1996 (BARB cases) in Great Britain Veterinary Record doi:10.1136/vr.100097
http://madcowfeed.blogspot.com/2012/03/case-control-study-of-cases-of-bovine.html
Wednesday, March 7, 2012
The epidemiology of bovine spongiform encephalopathy in the Republic of Ireland before and after the reinforced feed ban
http://madcowfeed.blogspot.com/2012/03/epidemiology-of-bovine-spongiform.html
Thursday, February 23, 2012
EIGHT FORMER SECRETARIES OF AGRICULTURE SPEAKING AT USDA'S 2012 AGRICULTURE OUTLOOK FORUM INDUCTED INTO USA MAD COW HALL OF SHAME
http://madcowusda.blogspot.com/2012/02/eight-former-secretaries-of-agriculture.html
Tuesday, February 14, 2012
White House budget proposes cuts to ag programs including TSE PRION disease aka mad cow type disease
http://transmissiblespongiformencephalopathy.blogspot.com/2012/02/white-house-budget-proposes-cuts-to-ag.html
Thursday, February 16, 2012
Bovine Spongiform Encephalopathy BSE
31 USA SENATORS ASK PRESIDENT OBAMA TO HELP SPREAD MAD COW DISEASE 2012
http://transmissiblespongiformencephalopathy.blogspot.com/2012/02/bovine-spongiform-encephalopathy-bse-31.html
> > > Ackerman says downed cattle are 50 times more likely to have mad cow disease (also known as Bovine Spongiform Encephalopathy, or BSE) than ambulatory cattle that are suspected of having BSE. Of the 20 confirmed cases of mad cow disease in North America since 1993, at least 16 have involved downer cattle, he said. < < <
don’t forget the children...
PLEASE be aware, for 4 years, the USDA fed our children all across the Nation (including TEXAS) dead stock downer cows, the most high risk cattle for BSE aka mad cow disease and other dangerous pathogens.
who will watch our children for CJD for the next 5+ decades ???
WAS your child exposed to mad cow disease via the NSLP ???
SCHOOL LUNCH PROGRAM FROM DOWNER CATTLE UPDATE
http://downercattle.blogspot.com/2009/05/who-will-watch-children.html
http://downercattle.blogspot.com/
DID YOUR CHILD CONSUME SOME OF THESE DEAD STOCK DOWNER COWS, THE MOST HIGH RISK FOR MAD COW DISEASE ???
you can check and see here ;
http://www.fns.usda.gov/fns/safety/pdf/Hallmark-Westland_byState.pdf
Wednesday, March 14, 2012
PINK SLIME, MRM’s, BSE AKA MAD COW DISEASE, AND THE USDA NSLP
http://madcowusda.blogspot.com/2012/03/pink-slime-mrms-bse-aka-mad-cow-disease.html
Saturday, March 5, 2011
MAD COW ATYPICAL CJD PRION TSE CASES WITH CLASSIFICATIONS PENDING ON THE RISE IN NORTH AMERICA
http://transmissiblespongiformencephalopathy.blogspot.com/2011/03/mad-cow-atypical-cjd-prion-tse-cases.html
Sunday, February 12, 2012
National Prion Disease Pathology Surveillance Center Cases Examined1 (August 19, 2011) including Texas
http://transmissiblespongiformencephalopathy.blogspot.com/2012/02/national-prion-disease-pathology.html
Thursday, March 01, 2012
Variant Creutzfeldt-Jakob disease Fact sheet N°180 Revised February 2012 W.H.O.
http://vcjd.blogspot.com/2012/03/variant-creutzfeldt-jakob-disease-fact.html
Friday, March 09, 2012
Experimental H-type and L-type bovine spongiform encephalopathy in cattle: observation of two clinical syndromes and diagnostic challenges
Research article
http://bse-atypical.blogspot.com/2012/03/experimental-h-type-and-l-type-bovine.html
Friday, December 23, 2011
Oral Transmission of L-type Bovine Spongiform Encephalopathy in Primate Model
Volume 18, Number 1—January 2012 Dispatch
http://transmissiblespongiformencephalopathy.blogspot.com/2011/12/oral-transmission-of-l-type-bovine.html
Thursday, March 15, 2012
Another cow enters food supply without being tested for BSE aka mad cow disease UK 15 March 2012
http://transmissiblespongiformencephalopathy.blogspot.com/2012/03/another-cow-enters-food-supply-without.html
Sunday, March 11, 2012
APHIS Proposes New Bovine Spongiform Encephalopathy Import Regulations in Line with International Animal Health Standards Proposal Aims to Ensure Health of the U.S. Beef Herd, Assist in Negotiations
while at the same time, reducing safety for humans via BSE aka mad cow type disease in North America and abroad via the OIE USDA trading of all strains of TSE prion disease via the BSE MRR policy $$$
http://transmissiblespongiformencephalopathy.blogspot.com/2012/03/aphis-proposes-new-bovine-spongiform.html
THANKS TO THE USDA AND THE OIE ET AL, they expose you and your children to mad cow type disease, because they refuse to go by their own science. see their junk science and policy there from ;
Research Project: STUDY OF ATYPICAL BSE Location: Virus and Prion Research Unit
Project Number: 3625-32000-086-05 Project Type: Specific Cooperative Agreement
Start Date: Sep 15, 2004 End Date: Sep 14, 2009
Objective: The objective of this cooperative research project with Dr. Maria Caramelli from the Italian BSE Reference Laboratory in Turin, Italy, is to conduct comparative studies with the U.S. bovine spongiform encephalopathy (BSE) isolate and the atypical BSE isolates identified in Italy. The studies will cover the following areas: 1. Evaluation of present diagnostics tools used in the U.S. for the detection of atypical BSE cases. 2. Molecular comparison of the U.S. BSE isolate and other typical BSE isolates with atypical BSE cases. 3. Studies on transmissibility and tissue distribution of atypical BSE isolates in cattle and other species.
Approach: This project will be done as a Specific Cooperative Agreement with the Italian BSE Reference Laboratory, Istituto Zooprofilattico Sperimentale del Piemonte, in Turin, Italy. It is essential for the U.S. BSE surveillance program to analyze the effectiveness of the U.S diagnostic tools for detection of atypical cases of BSE. Molecular comparisons of the U.S. BSE isolate with atypical BSE isolates will provide further characterization of the U.S. BSE isolate. Transmission studies are already underway using brain homogenates from atypical BSE cases into mice, cattle and sheep. It will be critical to see whether the atypical BSE isolates behave similarly to typical BSE isolates in terms of transmissibility and disease pathogenesis. If transmission occurs, tissue distribution comparisons will be made between cattle infected with the atypical BSE isolate and the U.S. BSE isolate. Differences in tissue distribution could require new regulations regarding specific risk material (SRM) removal.
http://www.ars.usda.gov/research/projects/projects.htm?accn_no=408490
3.Progress Report This report documents research conducted under a Specific Cooperative Agreement between ARS and the IST ZOOPROFIL SPERIMENT PIEMONTE. Additional details for the research can be found in the report for the parent project 3625-32000-086-00D, TRANSMISSION, DIFFERENTIATION, AND PATHOBIOLOGY OF TRANSMISSIBLE SPONGIFORM ENCEPHALOPATHIES
The aim of the cooperative research project was to: 1. Evaluate present diagnostic tools used in the U.S. for the detection of atypical bovine spongiform encephalopathy (BSE) cases. 2. Perform molecular comparison of the U.S. BSE isolate and other typical BSE isolates with atypical BSE cases. 3. Support studies on transmissibility and tissue distribution of atypical BSE isolates in cattle and other species. To complete objectives 1 and 2 (i.e., to compare Italian and U.S. BSE confirmatory protocols for detection of classical (C-) and atypical (H- and L-type) BSE cases), samples of Italian C-BSE and Italian L-type BSE (BASE), both frozen and formalin fixed, were sent to USDA laboratories in Ames, Iowa, to undergo Western blot (WB) and immunohistochemistry (IHC) comparison studies for PrPSc detection according to U.S. and Italian methods. A Western blot expert from the cooperating Italian lab assisted ARS scientists in performing the protocols from each laboratory in parallel. A comparative IHC study between U.S. and Italian BSE confirmatory protocols was also performed when the collaborator sent a scientist to Ames to assist in performing the Italian IHC protocol on BSE samples chosen for the study. Results obtained showed the Italian and U.S. IHC procedures were alike in PrPSc detection regarding its tissue distribution, deposition pattern and intensity of staining on all the C-, L- and H-type BSE cases considered. In addition, the U.S. protocol evidenced the characteristic presence of plaques in the frontal cortex of the Italian BASE case similar to the Italian protocol. Data from studies on objectives 1 & 2 has been presented at several international meetings in 2008 and 2009, and has been finalized into manuscript form for publication in a peer-reviewed journal (Journal of Veterinary Diagnostic Investigation). In support of objective 3, the cooperators completed and published their transmissibility and tissue distribution work on BASE cases in a peer-reviewed journal in 2008 (PLoS Pathogens Volume 4, page e1000075). They reported that in all experimentally infected atypical BSE animals, no PrPSc was detected in peripheral tissues either by standard Western blot analysis or following phosphotungstic acid precipitation. Peripheral issues examined included cervical and mesenteric lymph nodes, spleen, thymus, liver, lung, peripheral nerves, and forelimb and hind limb muscles. These findings support the conclusion there is no scientific evidence to expand the list of tissues included in the Specified Risk Material ban based on atypical BSE research data, thus confirming other studies indicating the pathogenesis of BSE in cattle is fundamentally different from that in sheep and mice, due to an exclusive intraneuronal spread of infectivity from the gut to the central nervous system. Methods used for monitoring included email, site visits, and periodic written reports.
http://www.ars.usda.gov/research/projects/projects.htm?ACCN_NO=408490&showpars=true&fy=2010
THE USDA AND THE OIE are industry friendly groups, tied to the hips for one reason, TRADE $$$
THERE is nothing science based about policy making for the TSE prion disease with neither one of these two groups i.e. OIE and the USDA.
IN MY OPINION, both organizations should be shut down for good, with an International Tribunal for their gross negligence with the TSE prion disease, thus, exposing humans and animals around the globe.
HOW many more will die ?
Neither the OIE or the USDA care about a slow incubating disease (as long as 50 years in some cases), that is 100% fatal, due to the fact that since the long incubation period, there is no way to trace back source.
I assure you, both the OIE and the USDA plan to keep it that way. ...
DEEP THROAT TO TSS 2000-2001 (take these old snips of emails with how ever many grains of salt you wish. ...tss)
The most frightening thing I have read all day is the report of Gambetti's finding of a new strain of sporadic cjd in young people...Dear God, what in the name of all that is holy is that!!! If the US has different strains of scrapie.....why????than the UK...then would the same mechanisms that make different strains of scrapie here make different strains of BSE...if the patterns are different in sheep and mice for scrapie.....could not the BSE be different in the cattle, in the mink, in the humans.......I really think the slides or tissues and everything from these young people with the new strain of sporadic cjd should be put up to be analyzed by many, many experts in cjd........bse.....scrapie Scrape the damn slide and put it into mice.....wait.....chop up the mouse brain and and spinal cord........put into some more mice.....dammit amplify the thing and start the damned research.....This is NOT rocket science...we need to use what we know and get off our butts and move....the whining about how long everything takes.....well it takes a whole lot longer if you whine for a year and then start the research!!! Not sure where I read this but it was a recent press release or something like that: I thought I would fall out of my chair when I read about how there was no worry about infectivity from a histopath slide or tissues because they are preserved in formic acid, or formalin or formaldehyde.....for God's sake........ Ask any pathologist in the UK what the brain tissues in the formalin looks like after a year.......it is a big fat sponge...the agent continues to eat the brain ......you can't make slides anymore because the agent has never stopped........and the old slides that are stained with Hemolysin and Eosin......they get holier and holier and degenerate and continue...what you looked at 6 months ago is not there........Gambetti better be photographing every damned thing he is looking at.....
Okay, you need to know. You don't need to pass it on as nothing will come of it and there is not a damned thing anyone can do about it. Don't even hint at it as it will be denied and laughed at.......... USDA is gonna do as little as possible until there is actually a human case in the USA of the nvcjd........if you want to move this thing along and shake the earth....then we gotta get the victims families to make sure whoever is doing the autopsy is credible, trustworthy, and a saint with the courage of Joan of Arc........I am not kidding!!!! so, unless we get a human death from EXACTLY the same form with EXACTLY the same histopath lesions as seen in the UK nvcjd........forget any action........it is ALL gonna be sporadic!!!
And, if there is a case.......there is gonna be every effort to link it to international travel, international food, etc. etc. etc. etc. etc. They will go so far as to find out if a sex partner had ever traveled to the UK/europe, etc. etc. .... It is gonna be a long, lonely, dangerous twisted journey to the truth. They have all the cards, all the money, and are willing to threaten and carry out those threats....and this may be their biggest downfall...
Thanks as always for your help. (Recently had a very startling revelation from a rather senior person in government here..........knocked me out of my chair........you must keep pushing. If I was a power person....I would be demanding that there be a least a million bovine tested as soon as possible and agressively seeking this disease. The big players are coming out of the woodwork as there is money to be made!!! In short: "FIRE AT WILL"!!! for the very dumb....who's "will"! "Will be the burden to bare if there is any coverup!"
again it was said years ago and it should be taken seriously....BSE will NEVER be found in the US! As for the BSE conference call...I think you did a great service to freedom of information and making some people feign integrity...I find it scary to see that most of the "experts" are employed by the federal government or are supported on the "teat" of federal funds. A scary picture! I hope there is a confidential panel organized by the new government to really investigate this thing.
You need to watch your back........but keep picking at them.......like a buzzard to the bone...you just may get to the truth!!! (You probably have more support than you know. Too many people are afraid to show you or let anyone else know. I have heard a few things myself... you ask the questions that everyone else is too afraid to ask.)
END...TSS
IN CONFIDENCE
Perceptions of unconventional slow virus in the USA
GAH WELLS
Report of a visit to the U.S.A. April-May 1989
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 fantical incident to be avoided in the USA AT ALL COSTS.
http://web.archive.org/web/20060307063531/http://www.bseinquiry.gov.uk/files/mb/m11b/tab01.pdf
and they meant it !
TSS
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