Greetings, 
Once again I must disclose my sincere disgust with the O.I.E. 
ONCE again the OIE has proven itself to be nothing more than a National  Brokerage Organization for all strains of Transmissible Spongiform  Encephalopathy. A failed organization, one that’s only purpose is to protect  trade.
OIE SEEMS TO HAVE nothing to do with protecting human or animal health, in  my opinion. 
OIE has proven this time and time with relations to animal Transmissible  Spongiform Encephalopathy TSE Prion disease typical and atypical. 
By the time the OIE recognizes a disease such as TSE as a threat to humans  or animals, it’s years, decades too late. 
THIS is the exact same thing that happened with Bovine Spongiform  Encephalopathy BSE TSE prion disease. Because of the OIE and it’s BSE  regulations and testing, BSE spread around the globe. 
This will happen with CWD in cervids, as it did with BSE in cattle and  Scrapie in sheep, especially and so foolishly for exempting the atypical TSE  prion disease, all because of trade and the almighty dollar. 
The OIE is nothing more than a ship of fools in my opinion, who’s absolute  purpose is to protect trade, and the almighty dollar, to hell with human and  animal health. 
IN terms of BSE TSE prion disease and the OIE, there never was no  ‘prevention’, it was simply never test enough to find.
This manual proves once again just this, in my opinion. ... 
 OIE Training Manual on Wildlife Diseases and Surveillance 
TO REDUCE TESTING OF BSE IN THE USA TO ONLY 40,000 A YEAR, is simply not  scientific regardless of what the OIE BSE testing protocol calls for. ALL one  has to do is look at the countries above that all went down with BSE, that all  went by the infamous OIE BSE testing protocols. THEN and only then, after the  USA finally fumbled the 'BSE FREE' golden egg and accidently had to document a  case or two of mad cow, low and behold, what next? yep, you guessed it, time to  move the goal post in the middle of the football game, GWs and his sleeping  partners at the OIE, gave birth to the BSE MRR policy, the legal trading of all  strains of TSE globally was born. ... 
SNIP... 
9/13/2005
Page 12 of 17
SEE SLIDES IN PDF FILE;
4. WHAT does USDA/FDA ET AL intend to do about the risks of atypical  BSE/TSE in cattle now that infectivity shows in tissue samples other than CNS in  Japan, the fact now that the last Texas mad cow and that last mad cow in Alabama  were indeed of the atypical strain, the fact that the studies long ago in  Mission, Texas of USA sheep scrapie transmission to the USA bovine, which proved  an 'atypical tse' in the USA bovine, the fact also that USDA/FDA are still  floundering on the last SRM regulations, but with the BASE strain now in cattle  that is not similar to nvCJD, but very similar to the sporadic CJD, and sporadic  CJD has tripled in the last few years in the USA. WHAT do you plan to do to  protect human health from these atypical strains of TSE, in relations to SRMs  ?
5. THE 2004 Enhanced BSE surveillance program, that tested all those cows,  but then we found just how terribly flawed the program was, from testing  protocols, to testing the most likely to have BSE i.e. high risk, to the  geographical distribution of the testing and high risk areas, to letting the  tissue samples of one mad cow sit on a shelf for 7+ months and then having to  have an act of Congress to ever get that cow finally confirmed, to that other  Texas mad cow they decided to not even bother testing at all, just rendered that  very suspect cow, to suspect to test evidently, back to that Alabama mad cow  that they could only give a guess as to age with dentition where we all know  that the age of that cow was so close to 10 years it could have been 9 years 7  months to 10 years 3 months, thus possibly being an BAPB i.e. USA 'born after  partial ban', to all those rabies suspect cows that did not have rabies, and DID  NOT get tested for BSE/TSE in that June 2004 enhanced surveillance program, even  though the common lay person knows the suspect rabies negative cows are suppose  to be BSE/TSE tested, how does one correct all these blatant failures and will  they be corrected? 
IT never was about human/animal health, but all about commodities and  futures. ... MISSION ACCOMPLISHED $$$ 
ENFAMOUS NON-SPECIES CODING SYSTEM BY FDA ET AL, another handy tool for  importing/exporting all strains of TSE ; 
Docket Management
Docket: 02N-0276 - Bioterrorism Preparedness; Registration of Food  Facilities, Section 305
Comment Number: EC –254
Accepted - Volume 11
Page 94 of 98
8/3/2006
ONE FINAL THOUGHT ;
OPINION
> > > New methodology, under the auspices of the OIE, is under  construction within the EU and EFSA and the Panel recommended that once these  classifications had been finalised they should harmonised with those used in the  EFSA BSE QRA guidance document. The Panel anticipated that this harmonisation  may have a knock-on impact on the QRA calculations, conclusions and  recommendations and that, again, future Panel members should review this, and  other, inputs of the QRA and address this impact using their “self-tasking  mandate” option. < < < 
GOD HELP US! 
sample survey via oie for bse is about 400 test via 100 million cattle, if  i am not mistaken. MOST countries that went by these OIE guidelines all  eventually went down with BSE. ...TSS 
 THE OIE has now shown they are nothing more than a National Trading  Brokerage for all strains of animal TSE. AS i said before, OIE should hang up  there jock strap now, since it appears they will buckle every time a country  makes some political hay about trade protocol, commodities and futures. IF they  are not going to be science based, they should do everyone a favor and dissolve  there organization. ... 
 Page 95 of 98
8/3/2006
WHAT ABOUT RISK FACTORS TO HUMANS FROM ALL OTHER TSEs, WITH RELATIONS TO  SRMs ???
a.. BSE OIE 
SNIP...SEE FULL TEXT ; 
 Transmissible Spongiform Encephalopathy and the O.I.E. 
 OIE Terrestrial Animal Health Standards Commission / September 2010 
 The EU takes note of the fact that atypical scrapie is not an OIE listed  disease. Nevertheless, it will remain notifiable in the EU. Moreover it must be  stressed that any emergence of this disease should be notified to the OIE by  Members and that scientific data should continue to be gathered. 
 snip... 
 Zoonotic Potential 
 Has transmission to humans been proven? (with the exception of artificial  circumstances) AND
Is human infection associated with severe consequences? (death or prolonged  illness) 
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. 
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) 
 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.
 ===================== 
"The origin of atypical BSE is not yet determined. According to EFSA's  scientific opinion published in 2008, all the cases of atypical BSE were  reported with birth dates before the real feed ban in January 2001 in Europe.  Therefore, the possibility of those atypical cases attributing to the  contaminated feeds, just as in classical BSE, cannot be completely denied."  
===================== 
atypical BSE TSE cases have been around for a long long time, so yes indeed  MBM or SRM could very well and most likely did contain atypical TSE of all  strains in the USA, and the typical strains as well, of all species. the North  America and the USA has typical and atypical BSE, typical and atypical Scrapie,  and two strains now of Chronic Wasting Disease, call them typical and atypical,  or call them what you want, all this has been rendered and fed to food producing  livestock for humans and animals for years in the USA. these are the facts, like  them or not. please see ; 
1992
IN CONFIDENCE
BSE - ATYPICAL LESION DISTRIBUTION (RBSE 92-21367)
 1992
NEW BRAIN DISORDER
3. WHAT ABOUT REPORTS OF NEW FORM OF BSE ?
THE VETERINARY RECORD HAS PUBLISHED AN ARTICLE ON A NEW BRAIN DISORDER OF  CATTLE DISCOVERED THROUGH OUR CONTROL MEASURES FOR BSE. ALTHOUGH IT PRESENTS  SIMILAR CLINICAL SIGNS TO BSE THERE ARE MAJOR DIFFERENCES IN HISTOPATHOLOGY AND  INCUBATION PERIODS BETWEEN THE TWO. MUST EMPHASISE THAT THIS IS _NOT_ BSE.
4. IS THIS NEW BRAIN DISORDER A THREAT ?
WE DO NOT EVEN KNOW WHETHER THE AGENT OF THIS DISEASE IS TRANSMISSIBLE. IN  ANY CASE, CASES SO FAR IDENTIFIED HAD SHOWN SIMILAR SYMPTOMS TO THOSE OF BSE,  AND THEREFORE HAVE BEEN SLAUGHTERED AND INCINERATED, SO THAT IF A TRANSMISSIBLE  AGENT WERE INVOLVED IT WOULD HAVE BEEN ELIMINATED. ...
Tuesday, November 17, 2009
SEAC NEW RESULTS ON IDIOPATHIC BRAINSTEM NEURONAL CHROMATOLYSIS (IBNC) FROM  THE VETERINARY LABORATORIES AGENCY (VLA) SEAC 103/1
 NEW RESULTS ON IDIOPATHIC BRAINSTEM NEURONAL CHROMATOLYSIS 
"All of the 15 cattle tested showed that the brains had abnormally  accumulated PrP" 
2009 
''THE LINE TO TAKE'' ON IBNC $$$ 1995 $$$ 
1995 
page 9 of 14 ; 
30. The Committee noted that the results were unusual. the questioned  whether there could be coincidental BSE infection or contamination with scrapie.  Dr. Tyrell noted that the feeling of the committee was that this did not  represent a new agent but it was important to be prepared to say something  publicly about these findings. A suggested line to take was that these were  scientifically unpublishable results but in line with the policy of openness  they would be made publicly available and further work done to test their  validity. Since the BSE precautions were applied to IBNC cases, human health was  protected. Further investigations should be carried out on isolations from  brains of IBNC cases with removal of the brain and subsequent handling under  strict conditions to avoid the risk of any contamination. 
31. Mr. Bradley informed the Committee that the CVO had informed the CMO  about the IBNC results and the transmission from retina and he, like the  Committee was satisfied that the controls already in place or proposed were  adequate. ... snip... see full text 
http://web.archive.org/web/20030327015011/http://www.bseinquiry.gov.uk/files/yb/1995/06/21005001.pdf  
Wednesday, July 28, 2010 
Atypical prion proteins and IBNC in cattle DEFRA project code SE1796 FOIA  Final report 
Tuesday, November 02, 2010 
BSE - ATYPICAL LESION DISTRIBUTION (RBSE 92-21367) statutory (obex only)  diagnostic criteria CVL 1992 
Wednesday, February 16, 2011 
IN CONFIDENCE 
SCRAPIE TRANSMISSION TO CHIMPANZEES 
IN CONFIDENCE 
Sunday, April 18, 2010 
SCRAPIE AND ATYPICAL SCRAPIE TRANSMISSION STUDIES A REVIEW 2010 
 Monday, April 25, 2011 
Experimental Oral Transmission of Atypical Scrapie to Sheep 
Volume 17, Number 5-May 2011 
 Thursday, June 2, 2011 
USDA scrapie report for April 2011 NEW ATYPICAL NOR-98 SCRAPIE CASES  Pennsylvania AND California 
 Monday, June 20, 2011 2011 
Annual Conference of the National Institute for Animal Agriculture ATYPICAL  NOR-98 LIKE SCRAPIE UPDATE USA 
 Thursday, July 14, 2011 
Histopathological Studies of "CH1641-Like" Scrapie Sources Versus Classical  Scrapie and BSE Transmitted to Ovine Transgenic Mice (TgOvPrP4) 
 Wednesday, October 12, 2011 
White-tailed deer are susceptible to the agent of sheep scrapie by  intracerebral inoculation 
 Saturday, December 18, 2010 
 OIE Global Conference on Wildlife Animal Health and Biodiversity –  Preparing for the Future (TSE AND PRIONS) Paris (France), 23-25 February 2011  
 I see again that the OIE has done little to help eradicate all animal TSE  from the globe, and in fact in my opinion, have help enhance the spread of BSE  and other animal TSE globally by their industry friendly regulations. I tried to  warn the OIE in 2002 about CWD and the potential, but very real threat of CWD to  humans. I was told that they were seriously considering this. what happened ?  NOW, the OIE and the USDA collaborate to make legal the trading of all strains  of atypical BSE legal, and in fact have done so with the atypical scrapie, when  science has made perfectly clear the risk factors to humans and other species. I  have said it once (see below), and i will say again ; 
 "THE OIE has now shown they are nothing more than a National Trading  Brokerage for all strains of animal TSE. AS i said before, OIE should hang up  there jock strap now, since it appears they will buckle every time a country  makes some political hay about trade protocol, commodities and futures. IF they  are not going to be science based, they should do everyone a favor and dissolve  there organization." 
 JUST about every country that went by the infamous O.I.E. B.S.E.  guidelines, most all came down with B.S.E. ...TSS 
 NOW, some history on the failed OIE BSE/TSE policy, and why the OIE  allowed BSE and other TSE to spread around the globe $$$ 
 SNIP... 
 i proposed to OIE years ago to include CWD. but with these new atypical  case of TSE showing up in cattle and sheep, it will be interesting to see how  the OIE handles the USA demands on weakening the BSE/TSE regs for exporting  countries; 
 Date: Fri, 12 Jul 2002 16:11:42 –0700 
 Reply-To: B S E-l 
 Sender: Bovine Spongiform Encephalopathy 
 From: TSS 
 Subject: CWD/USA — CWD/OIE? 
 snip... 
 Greetings List Members, 
 speaking with someone at the OIE about my concerns with CWD and the  non-testing for TSEs in USA cattle, i find it very sad that the OIE does not  follow CWD related issues. BUT, they voice my same concerns and said changes are  in the makings. sadly, the changes will take about 2 years? 
 snip... 
 ''I agree with you Dr Terry. The OIE, namely the International Animal  Health Code Commission is working on making proposals to Member Countries to  change the OIE lists so to avoid some the problems mentioned in you e-mail. This  will take at least two years before adoption by the International Committee.''  
 snip... 
 two years is a very long time, on an issue of such importance to both  humans and animals... 
 kind regards, terry 
snip... 
PAGE 25 Transmission Studies Mule deer transmissions of CWD were by  intracerebral inoculation and compared with natural cases resulted in a more  rapidly progressive clinical disease with repeated episodes of synocopy ending  in coma. One control animal became affected, it is believed through  contamination of inoculam (?saline). Further CWD transmissions were carried out  by Dick Marsh into ferret, mink and squirrel monkey. Transmission occurred in  all of these species with the shortest incubation period in the ferret...
Clearly, it is premature to draw firm conclusions about CWD passing  naturally into humans, cattle and sheep, but the present results suggest that  CWD transmissions to humans would be as limited by PrP incompatibility as  transmissions of BSE or sheep scrapie to humans. Although there is no evidence  that sheep scrapie has affected humans, it is likely that BSE has caused variant  CJD in 74 people (definite and probable variant CJD cases to date according to  the UK CJD Surveillance Unit). Given the presumably large number of people  exposed to BSE infectivity, the susceptibility of humans may still be very low  compared with cattle, which would be consistent with the relatively inefficient  conversion of human PrP-sen by PrPBSE. Nonetheless, since humans have apparently  been infected by BSE, it would seem prudent to take reasonable measures to limit  exposure of humans (as well as sheep and cattle) to CWD infectivity as has been  recommended for other animal TSEs.
snip...
and why do we not want to do TSE transmission studies on chimpanzees $  
snip... 
 5. A positive result from a chimpanzee challenged severly would likely  create alarm in some circles even if the result could not be interpreted for  man. I have a view that all these agents could be transmitted provided a large  enough dose by appropriate routes was given and the animals kept long enough.  Until the mechanisms of the species barrier are more clearly understood it might  be best to retain that hypothesis. 
 snip... 
 R. BRADLEY 
same reason CJD/TSE is not reportable Nationally in the USA. same reason no  CJD questionnaire exists in the USA that is issued to all victims and families  of victims asking real questions pertaining to route and source of agent. no  autopies for all demented of young AND OLD! same reason the USA is steadfast  refusing to this day to rapid TSE test all cattle for human/animal consumption.  the USA simply does not want to know$ 
 hell, we should just retain it all, and just play like it has not happened  for the next 40 years as well. hmm, something else to ponder ; 
 5. A positive result from a chimpanzee challenged severly would likely  create alarm in some circles even if the result could not be interpreted for man  
 so, when/where is our first case transmission study of TSE on man going to  be? i wish to witness this and have a few suggestions for our first human  guinea-pigs ;-) 
 Terry S. Singeltary Sr. P.O. Box 42 Bacliff, TEXAS USA 77518 
END...TSS 
 C H A P T E R 2 . 3 . 1 3 .
BOVINE SPONGIFORM ENCEPHALOPATHY
Article 2.3.13.1.
SNIP...please see full text ;
putting the cart before the horse OIE TSE policy. ...TSS 
Monday, November 30, 2009
USDA AND OIE COLLABORATE TO EXCLUDE ATYPICAL SCRAPIE NOR-98 ANIMAL HEALTH  CODE
Friday, February 11, 2011
Atypical/Nor98 Scrapie Infectivity in Sheep Peripheral Tissues
Sunday, December 12, 2010
EFSA reviews BSE/TSE infectivity in small ruminant tissues News Story 2  December 2010
Thursday, December 23, 2010
Molecular Typing of Protease-Resistant Prion Protein in Transmissible  Spongiform Encephalopathies of Small Ruminants, France, 2002–2009 Volume 17,  Number 1–January 2011
Thursday, November 18, 2010
Increased susceptibility of human-PrP transgenic mice to bovine spongiform  encephalopathy following passage in sheep
Sunday, October 3, 2010
Scrapie, Nor-98 atypical Scrapie, and BSE in sheep and goats North America,  who's looking ?
Saturday, January 29, 2011
Atypical L-Type Bovine Spongiform Encephalopathy (L-BSE) Transmission to  Cynomolgus Macaques, a Non-Human Primate
Jpn. J. Infect. Dis., 64 (1), 81-84, 2011
Thursday, February 10, 2011
Chronic Wasting Disease Found In A White-Tailed Deer In Maryland
CWD Maryland Emergency Response Plan 2011
Thursday, February 10, 2011
CWD ILLINOIS UPDATE FEBRUARY 2011 Locations of CWD-Positive Deer – Updated  2/07/2011
Wednesday, February 09, 2011
CWD Minnesota deer feeding ban covering Dodge, Goodhue, Olmsted, and  Wabasha counties will become effective Feb. 14, 2011
Tuesday, January 25, 2011
Minnesota, National Veterinary Services Laboratory in Ames, Iowa, has  confirmed CWD case near Pine Island
UPDATED DATA ON 2ND CWD STRAIN
Wednesday, September 08, 2010
CWD PRION CONGRESS SEPTEMBER 8-11 2010
Tuesday, January 25, 2011
Generation of a new form of human PrPSc in vitro by inter-species  transmission from cervids prions
Our results have far-reaching implications for human health, since they  indicate that cervid PrPSc can trigger the conversion of human PrPC into PrPSc,  suggesting that CWD might be infectious to humans. Interestingly our findings  suggest that unstable strains from CWD affected animals might not be a problem  for humans, but upon strain stabilization by successive passages in the wild,  this disease might become progressively more transmissible to man.
Reference List
snip...
please see full text and many thanks to the Professor Soto and the other  Authors of this study AND to The Journal Of Biological Chemistry for the free  full text !!!
Tuesday, January 25, 2011
Generation of a new form of human PrPSc in vitro by inter-species  transmission from cervids prions
PLEASE NOTE ;
there are now two documented strains of CWD, and science is showing that  indeed CWD could transmit to humans via transmission studies ; 
 P35
ADAPTATION OF CHRONIC WASTING DISEASE (CWD) INTO HAMSTERS, EVIDENCE OF A  WISCONSIN STRAIN OF CWD
Chad Johnson1, Judd Aiken2,3,4 and Debbie McKenzie4,5 1 Department of  Comparative Biosciences, University of Wisconsin, Madison WI, USA 53706 2  Department of Agriculture, Food and Nutritional Sciences, 3 Alberta Veterinary  Research Institute, 4.Center for Prions and Protein Folding Diseases, 5  Department of Biological Sciences, University of Alberta, Edmonton AB, Canada  T6G 2P5
The identification and characterization of prion strains is increasingly  important for the diagnosis and biological definition of these infectious  pathogens. Although well-established in scrapie and, more recently, in BSE,  comparatively little is known about the possibility of prion strains in chronic  wasting disease (CWD), a disease affecting free ranging and captive cervids,  primarily in North America. We have identified prion protein variants in the  white-tailed deer population and demonstrated that Prnp genotype affects the  susceptibility/disease progression of white-tailed deer to CWD agent. The  existence of cervid prion protein variants raises the likelihood of distinct CWD  strains. Small rodent models are a useful means of identifying prion strains. We  intracerebrally inoculated hamsters with brain homogenates and phosphotungstate  concentrated preparations from CWD positive hunter-harvested (Wisconsin CWD  endemic area) and experimentally infected deer of known Prnp genotypes. These  transmission studies resulted in clinical presentation in primary passage of  concentrated CWD prions. Subclinical infection was established with the other  primary passages based on the detection of PrPCWD in the brains of hamsters and  the successful disease transmission upon second passage. Second and third  passage data, when compared to transmission studies using different CWD inocula  (Raymond et al., 2007) indicate that the CWD agent present in the Wisconsin  white-tailed deer population is different than the strain(s) present in elk,  mule-deer and white-tailed deer from the western United States endemic  region.
PPo3-7:
Prion Transmission from Cervids to Humans is Strain-dependent
Qingzhong Kong, Shenghai Huang,*Fusong Chen, Michael Payne, Pierluigi  Gambetti and Liuting Qing Department of Pathology; Case western Reserve  University; Cleveland, OH USA *Current address: Nursing Informatics; Memorial  Sloan-Kettering Cancer Center; New York, NY USA
Key words: CWD, strain, human transmission
Chronic wasting disease (CWD) is a widespread prion disease in cervids  (deer and elk) in North America where significant human exposure to CWD is  likely and zoonotic transmission of CWD is a concern. Current evidence indicates  a strong barrier for transmission of the classical CWD strain to humans with the  PrP-129MM genotype. A few recent reports suggest the presence of two or more CWD  strains. What remain unknown is whether individuals with the PrP-129VV/MV  genotypes are also resistant to the classical CWD strain and whether humans are  resistant to all natural or adapted cervid prion strains. Here we report that a  human prion strain that had adopted the cervid prion protein (PrP) sequence  through passage in cervidized transgenic mice efficiently infected transgenic  mice expressing human PrP, indicating that the species barrier from cervid to  humans is prion strain-dependent and humans can be vulnerable to novel cervid  prion strains. Preliminary results on CWD transmission in transgenic mice  expressing human PrP-129V will also be discussed.
Acknowledgement Supported by NINDS NS052319 and NIA AG14359. 
PPo2-27:
Generation of a Novel form of Human PrPSc by Inter-species Transmission of  Cervid Prions
Marcelo A. Barria,1 Glenn C. Telling,2 Pierluigi Gambetti,3 James A.  Mastrianni4 and Claudio Soto1 1Mitchell Center for Alzheimer's disease and  related Brain disorders; Dept of Neurology; University of Texas Houston Medical  School; Houston, TX USA; 2Dept of Microbiology, Immunology & Molecular  Genetics and Neurology; Sanders Brown Center on Aging; University of Kentucky  Medical Center; Lexington, KY USA; 3Institute of Pathology; Case western Reserve  University; Cleveland, OH USA; 4Dept of Neurology; University of Chicago;  Chicago, IL USA
Prion diseases are infectious neurodegenerative disorders affecting humans  and animals that result from the conversion of normal prion protein (PrPC) into  the misfolded and infectious prion (PrPSc). Chronic wasting disease (CWD) of  cervids is a prion disorder of increasing prevalence within the United States  that affects a large population of wild and captive deer and elk. CWD is highly  contagious and its origin, mechanism of transmission and exact prevalence are  currently unclear. The risk of transmission of CWD to humans is unknown.  Defining that risk is of utmost importance, considering that people have been  infected by animal prions, resulting in new fatal diseases. To study the  possibility that human PrPC can be converted into the infectious form by CWD  PrPSc we performed experiments using the Protein Misfolding Cyclic Amplification  (PMCA) technique, which mimic in vitro the process of prion replication. Our  results show that cervid PrPSc can induce the pathological conversion of human  PrPC, but only after the CWD prion strain has been stabilized by successive  passages in vitro or in vivo. Interestingly, this newly generated human PrPSc  exhibits a distinct biochemical pattern that differs from any of the currently  known forms of human PrPSc, indicating that it corresponds to a novel human  prion strain. Our findings suggest that CWD prions have the capability to infect  humans, and that this ability depends on CWD strain adaptation, implying that  the risk for human health progressively increases with the spread of CWD among  cervids. 
PPo2-7:
Biochemical and Biophysical Characterization of Different CWD  Isolates
Martin L. Daus and Michael Beekes Robert Koch Institute; Berlin,  Germany
Key words: CWD, strains, FT-IR, AFM
Chronic wasting disease (CWD) is one of three naturally occurring forms of  prion disease. The other two are Creutzfeldt-Jakob disease in humans and scrapie  in sheep. CWD is contagious and affects captive as well as free ranging cervids.  As long as there is no definite answer of whether CWD can breach the species  barrier to humans precautionary measures especially for the protection of  consumers need to be considered. In principle, different strains of CWD may be  associated with different risks of transmission to humans. Sophisticated strain  differentiation as accomplished for other prion diseases has not yet been  established for CWD. However, several different findings indicate that there  exists more than one strain of CWD agent in cervids. We have analysed a set of  CWD isolates from white-tailed deer and could detect at least two biochemically  different forms of disease-associated prion protein PrPTSE. Limited proteolysis  with different concentrations of proteinase K and/or after exposure of PrPTSE to  different pH-values or concentrations of Guanidinium hydrochloride resulted in  distinct isolate-specific digestion patterns. Our CWD isolates were also  examined in protein misfolding cyclic amplification studies. This showed  different conversion activities for those isolates that had displayed  significantly different sensitivities to limited proteolysis by PK in the  biochemical experiments described above. We further applied Fourier transform  infrared spectroscopy in combination with atomic force microscopy. This  confirmed structural differences in the PrPTSE of at least two disinct CWD  isolates. The data presented here substantiate and expand previous reports on  the existence of different CWD strains. 
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. ...end 
 http://web.archive.org/web/20030511010117/http://www.bseinquiry.gov.uk/files/yb/1994/10/00003001.pdf  
UPDATED DATA ON 2ND CWD STRAIN
Wednesday, September 08, 2010
CWD PRION CONGRESS SEPTEMBER 8-11 2010
snip... see full text ; 
 Tuesday, January 25, 2011
Generation of a new form of human PrPSc in vitro by inter-species  transmission from cervids prions
 2011-2012 
 EFSA Journal 2011 The European Response to BSE: A Success Story 
This is an interesting editorial about the Mad Cow Disease debacle, and  it's ramifications that will continue to play out for decades to come ; 
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... 
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  http://transmissiblespongiformencephalopathy.blogspot.com/  
 Thursday, December 29, 2011 
Aerosols An underestimated vehicle for transmission of prion diseases?  
PRION www.landesbioscience.com 
 please see more on Aerosols and TSE prion disease here ; 
Saturday, December 31, 2011
Depopulation Plan Being Developed for Captive Deer Facility in Macon County  after second CWD positive confirmation 
please see this game farm that was shut down, and the incredible infection  rate ; 
Tuesday, December 20, 2011 
CHRONIC WASTING DISEASE CWD WISCONSIN Almond Deer (Buckhorn Flats) Farm  Update DECEMBER 2011 
Wednesday, December 21, 2011 
CWD UTAH San Juan deer hunting unit 
Monday, November 14, 2011 
WYOMING Creutzfeldt Jakob Disease, CWD, TSE, PRION REPORTING 2011 
Wednesday, November 16, 2011 
Wisconsin Creutzfeldt Jakob Disease, CWD, TSE, PRION REPORTING 2011 
Sunday, November 13, 2011 
COLORADO CWD CJD TSE PRION REPORTING 2011 
Monday, June 27, 2011 
Zoonotic Potential of CWD: Experimental Transmissions to Non-Human Primates  
Friday, December 23, 2011 
Oral Transmission of L-type Bovine Spongiform Encephalopathy in Primate  Model 
Volume 18, Number 1—January 2012 Dispatch 
Saturday, December 3, 2011 Isolation of Prion with BSE Properties from  Farmed Goat 
Volume 17, Number 12—December 2011 
COLLINGE THREATENS TO GO TO MEDIA 
http://web.archive.org/web/20040315075058/http://www.bseinquiry.gov.uk/files/yb/1992/12/16005001.pdf  
Wednesday, August 20, 2008 
Bovine Spongiform Encephalopathy Mad Cow Disease typical and atypical  strains, was there a cover-up ? SNIP... 
IN CONFIDENCE
This is a highly competitive field and it really will be a pity if we allow  many of the key findings to be published by overseas groups while we are unable  to pursue our research findings because of this disagreement, which I hope we  can make every effort to solve. 
http://web.archive.org/web/20040315054541/http://www.bseinquiry.gov.uk/files/yb/1992/10/26002001.pdf  
COLLINGE THREATENS TO GO TO MEDIA 
http://web.archive.org/web/20040315075058/http://www.bseinquiry.gov.uk/files/yb/1992/12/16005001.pdf  
2. The discovery might indicate the existence of a different strain of BSE  from that present in the general epidemic or an unusual response by an  individual host.
3. If further atypical lesion distribution cases are revealed in this herd  then implications of misdiagnosis of 'negative' cases in other herds may not be  insignificant.
snip...
This minute is re-issued with a wider distribution. The information  contained herein should NOT be disseminated further except on the basis of  ''NEED TO KNOW''.
R Bradley 
http://web.archive.org/web/20041226015613/http://www.bseinquiry.gov.uk/files/yb/1993/02/17001001.pdf  
IN CONFIDENCE 
 BSE ATYPICAL LESION DISTRIBUTION 
http://web.archive.org/web/20041226015813/http://www.bseinquiry.gov.uk/files/yb/1993/03/14001001.pdf  
 1983 
BSE CONSULTANT
APPROVAL OF MATERIAL FOR PUBLICATIONS
All material for publication including written works to be published in  scientific journals, books, proceedings of scientific meetings, abstracts of  verbally delivered papers and the like should be scrutinized for risk to the  Ministry before dispatch to the publishers.............
full text; 
http://web.archive.org/web/20041031210056/http://www.bseinquiry.gov.uk/files/yb/1983/10/12001001.pdf  
- 10 -
19. On 18th February, 1987 (YB87/2.18/1.1) I reported to Dr Watson and Dr  Shreeve on a further case which we had received from Truro VIC. The brain had  shown neuronal vacuolation and in brain extracts there were fibrils that were  similar in size and appearance to SAFs from sheep with scrapie. The Virology  Department was studying the brain further and considering a transmission study.  A few weeks before this, I had discussed the possibility of a transmission study  with Michael Dawson, a research officer in the Virology Department and an expert  in viral diseases in sheep, and we were considering carefully the safety  aspects. In my note I raised the question of whether we should disclose the  information we had more widely to the VIS because this may assist in getting any  other cases referred to CVL but there was the difficulty that we knew very  little about the disorder and would be unable to deal with queries that might be  raised.
20. On 23rd February, 1987 (YB87/2.23/1.1) I sent Mr Wells a note asking  him to prepare a statement for publication in Vision, the in-house newsheet  prepared by the VIS for the SVS, setting out details of what we had discovered.  On 24th February, 1987 (YB87/2.25/2.1) Gerald Wells indicated in a note to me  that he had discussed the proposed article with Mr Dawson and they both believed  that it could be damaging to publish anything at that stage. They believed cases  would be referred to CVL in any event because they were unusual and they did not  feel "Vision" was an appropriate publication because its confidentiality was  questionable and might lead to referrals to veterinary schools rather than CVL.  Gerald Wells was also concerned about the resources available in his section to  deal with referred cases. I replied (YB87/2.25/2.1) indicating a draft statement  was needed by the Director before a decision on publication could be made.  Gerald Wells prepared a draft statement (YB87/3.2/2.1) and sent it to me on 2nd  March, 1987. In his cover note (YB87/3.2/1.1) he commented that he believed the  distribution of any statement about the new disease outside of CVL to be  premature because there was so little information available about the new  disease. I passed on a copy of Gerald Wells' note to Dr Watson (YB87/3.2/3.1). I  discussed the matter of publication with Dr Watson. No decision had been taken  to publish any material at that stage and I sent a note to Gerald Wells letting  him know the position and confirming that his views and those of Michael Dawson  would be taken into account when a decision was taken. 
- 11 -
21. In March, 1987 serious consideration was given to possible transmission  (e.g. to hamsters) and other experiments (other than the collection of  epidemiological data by the VIS and clinicopathology which had been in progress  since the first cases were recognised in November, 1986).
22. On 23rd April, 1987 I sent a report (YB87/4.23/1.1) to Dr Watson and Dr  Shreeve informing them that nine control brains were being examined for SAFs and  a cow which appeared to be affected with BSE had been purchased for observation.  The cow had come from the farm where the original cases had developed and had  arrived at CVL on 22nd April, 1987.
23. On 15th May, 1987 Dr Watson informed me that the proposed "Vision"  draft would be circulated to VICs in England and Wales if it was approved by  management. On 22nd May, 1987 I was copied in on a note (YB87/5.22/2.1) from B.M  Williams, (who I believe was Head of the VIS at this time but retired shortly  after this), to Dr Watson. This confirmed that the draft prepared for  publication in Vision was approved but that the final paragraph should be  amended to make it clear that knowledge of the new disease should not be  communicated to other research institutes or university departments. At a  meeting with Dr Watson on 2nd June, 1987 he informed me that no communication  should be made with NPU until after the meeting with the CVO on 5th June, 1987  (see my note of 3rd June, 1987 – YB87/6.3/1.1). We needed much more data and  information to answer inevitable queries. ... 
*This case study accompanies the IRGC report “Risk Governance Deficits: An  analysis and illustration of the most common deficits in risk governance”. The  Bovine Spongiform Encephalopathy (BSE) Epidemic in the United Kingdom
By Belinda Cleeland1
SNIP...
A6 Misrepresenting information about risk
From the very beginning of the BSE outbreak, not only was knowledge  misrepresented by the British government, but in some cases it was even  withheld. For example, after the initial diagnosis of BSE by the SVS in late  1986, there was an embargo placed on the sharing, or making public, of any  BSE-related information that ran until mid-1987. Also, up until at least 1990,  outside scientists that requested access to BSE data to conduct further studies  were denied, despite the fact the improved scientific understanding of the  disease had the greatest potential to minimise the impact of the epidemic. Even  government scientists within the CVL have acknowledged that there was a culture  of suppressing information, to the point that studies revealing damaging  evidence (e.g. that there was a causal link between BSE and the new  encephalopathy found in cats) were denied publication permission [Ashraf, 2000].  The withholding of such information allowed the government to publicly assert  that BSE was just like another version of scrapie – not transmissible to humans  – and that there was “clear scientific evidence that British beef is perfectly  safe” [UK House of Commons, 1990].2 This was certainly a misrepresentation of  the knowledge held at the time, and one that was only possible due to the  suppression of some scientific findings and recommendations. Of course, the main  reason for this misrepresentation of knowledge was the protection of  agricultural and industrial interests – the specific stakeholder favoured in  this case was the British beef industry, which stood to lose billions of pounds  if a large number of its animals had to be slaughtered, if export bans were put  in place, or if costly regulations were implemented. To protect the interests of  the beef industry, the government would assert on many occasions that British  beef was safe to eat and that regulatory controls already implemented would  prevent any 2 This comment was made by the Agriculture Minister to the House of  Commons.
contaminated material from entering the food chain. This was also a  misrepresentation of knowledge, as the government was fully aware that their  measures were not designed to eliminate exposure, but only to diminish the risk  [van Zwanenberg & Millstone, 2002:161].
What’s more, many uncertainties relating to the transmissibility of the  disease were either down-played or ignored, resulting in an overstatement of  certainty that British beef was completely safe to eat and that BSE was not  transmissible to humans. The way uncertainty was dealt with in this case was the  result of a number of factors, including the desire to protect specific  stakeholder interests. One crucial factor was the underlying element of risk  political culture in the UK that linked the identity of the actor to the  consistency of his policy positions. This led to consistency of position being  prioritised over accuracy [Dressel, 2000], and resulted in the government  insisting on the absence of risk to the population, maintaining this public  position despite mounting evidence to the contrary. Although aware of them,  policy-makers chose not to overtly acknowledge the levels of uncertainty and the  complexity of the risks involved with BSE and its spread because the  ramifications of these were too great for the interests they were trying to  safeguard.
B1 Responding to early warnings
The incorporation of rendered meat and bone meal into animal feed creates a  number of risks related to the transmission, recycling and amplification of  pathogens. Such risks were recognised well before the emergence of BSE. In the  US in the mid-1970s, concerns that scrapie may be linked to CJD (although there  is no evidence that scrapie is transmissible to humans) led to some regulations  being placed on the incorporation of sheep or goat carcasses into human and  animal foods [van Zwanenberg & Millstone, 2002:158]. In the UK, too, the  Royal Commission on Environmental Pollution recommended in 1979 that minimum  processing standards be implemented by the rendering industries in order to  minimise the potential for disease spread [RCEP, 1979]. The incoming Thatcher  government withdrew these proposed regulations, preferring to let industry  decide for itself what standards to use. In retrospect, the failure to act at  this point to mitigate the general risk of disease transmission may have had a  crucial impact on the later outbreak of BSE, given that the disease “probably  originated from a novel source in the early 1970s” [BSE Inquiry, 2000b]. Early  warnings that BSE might be transmissible to humans were, in fact, observed by  scientists and government officials throughout the period from 1986 (the time of  first diagnosis in cattle) to 1995 (when vCJD was first observed in humans).  Such observations are noted in, for example, the minutes of a meeting of the  National Institute for Biological Standards and Control in May 1988, where the  probability of transmission of BSE to humans is assessed as more than remote.  The diagnosis in 1990 of a domestic cat with a previously unknown spongiform  encephalopathy resembling BSE indicated that the disease could infect a wider  range of hosts. Responses to such early warnings of potential dangers to human  health were either too weak or came too late. This may have been partly a result  of an ‘unwillingness to know’ due to the economic harm this knowledge would  cause the UK beef industry (related to deficit A6); and partly due to  institutional capacities and procedures (related to deficits B5, 9 and  10).
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 $$$ 
Docket APHIS-2006-0041 Docket Title Bovine Spongiform Encephalopathy;  Minimal-Risk Regions; Importation of Live Bovines and Products Derived from  Bovines Commodities Docket Type Rulemaking Document APHIS-2006-0041-0001  Document Title Bovine Spongiform Encephalopathy; Minimal-Risk Regions;  Importation of Live Bovines and Products Derived From Bovines Public Submission  APHIS-2006-0041-0006 Public Submission Title Comment from Terry S Singletary Sr  Views Add Comments How To Comment
snip...
MY personal belief, since you ask, is that not only the Canadian border,  but the USA border, and the Mexican border should be sealed up tighter than a  drum for exporting there TSE tainted products, until a validated, 100% sensitive  test is available, and all animals for human and animal consumption are tested.  all we are doing is the exact same thing the UK did with there mad cow poisoning  when they exported it all over the globe, all the while knowing what they were  doing. this BSE MRR policy is nothing more than a legal tool to do just exactly  what the UK did, thanks to the OIE and GW, it's legal now. and they executed  Saddam for poisoning ???
go figure....
Terry S. Singeltary Sr. P.O. Box 42 Bacliff, Texas USA 77518 
Monday, January 2, 2012
EFSA Minutes of the 6th Meeting of the EFSA Scientific Network on BSE-TSE  Brussels, 29-30 November 2011 
 Thursday, January 05, 2012 
Comparative analysis of Japanese and foreign L-type BSE prions 
 Friday, December 23, 2011
Oral Transmission of L-type Bovine Spongiform Encephalopathy in Primate  Model 
Volume 18, Number 1—January 2012 Dispatch 
 Wednesday, May 25, 2011
O.I.E. Terrestrial Animal Health Standards Commission and prion (TSE)  disease reporting 2011 
----- Original Message ----- 
From: Terry S. Singeltary Sr. 
To: BSE-L@LISTS.AEGEE.ORG 
Cc: trade@oie.int ; oie@oie.int ; f.diaz@oie.int ; scientific.dept@oie.int  ; cjdvoice@yahoogroups.com ; BLOODCJD@YAHOOGROUPS.COM 
Sent: Tuesday, May 24, 2011 2:24 PM
Subject: O.I.E. Terrestrial Animal Health Standards Commission and prion  (TSE) disease reporting 2011
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" 
Saturday, November 19, 2011 
Novel Prion Protein in BSE-affected Cattle, Switzerland 
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... 
2011 Monday, September 26, 2011 
L-BSE BASE prion and atypical sporadic CJD 
Wednesday, March 31, 2010 
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.
 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...
Owens, Julie
From: Terry S. Singeltary Sr. [flounder9@verizon.net]
Sent: Monday, July 24, 2006 1:09 PM
To: FSIS RegulationsComments
Subject: [Docket No. FSIS-2006-0011] FSIS Harvard Risk Assessment of Bovine  Spongiform Encephalopathy (BSE)
Page 1 of 98
FSIS RFEPLY TO TSS ; 
Harvard Risk Assessment of Bovine Spongiform Encephalopathy Update, October  31, 2005 INTRODUCTION The United States Department of Agriculture’s Food Safety  and Inspection Service (FSIS) held a public meeting on July 25, 2006 in  Washington, D.C. to present findings from the Harvard Risk Assessment of Bovine  Spongiform Encephalopathy Update, October 31, 2005 (report and model located on  the FSIS website: 
Comments on technical aspects of the risk assessment were then submitted to  FSIS. Comments were received from Food and Water Watch, Food Animal Concerns  Trust (FACT), Farm Sanctuary, R-CALF USA, Linda A Detwiler, and Terry S.  Singeltary. This document provides itemized replies to the public comments  received on the 2005 updated Harvard BSE risk assessment. Please bear the  following points in mind: 
Saturday, June 19, 2010 
U.S. DENIED UPGRADED BSE STATUS FROM OIE 
 Friday, August 20, 2010 
USDA: Animal Disease Traceability August 2010 
 Friday, November 18, 2011 
country-of-origin labeling law (COOL) violates U.S. obligations under WTO  rules WT/DS384/R WT/DS386/R 
 Saturday, July 23, 2011 
CATTLE HEADS WITH TONSILS, BEEF TONGUES, SPINAL CORD, SPECIFIED RISK  MATERIALS (SRM's) AND PRIONS, AKA MAD COW DISEASE 
 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 
 Archive Number 20101206.4364 Published Date 06-DEC-2010 Subject  PRO/AH/EDR> 
Prion disease update 2010 (11) PRION DISEASE UPDATE 2010 (11) 
Terry S. Singeltary Sr. on the Creutzfeldt-Jakob Disease Public Health  Crisis
 full text with source references ; 
 TSS
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