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BOVINE SPONGIFORM ENCEPHALOPATHY BSE TSE PRION SURVEILLANCE, TESTING, AND SRM REMOVAL UNITED STATE OF AMERICA UPDATE JULY 2016

About Me

My mother was murdered by what I call corporate and political homicide i.e. FOR PROFIT! she died from a rare phenotype of CJD i.e. the Heidenhain Variant of Creutzfeldt Jakob Disease i.e. sporadic, simply meaning from unknown route and source. I have simply been trying to validate her death DOD 12/14/97 with the truth. There is a route, and there is a source. There are many here in the USA. WE must make CJD and all human TSE, of all age groups 'reportable' Nationally and Internationally, with a written CJD questionnaire asking real questions pertaining to route and source of this agent. Friendly fire has the potential to play a huge role in the continued transmission of this agent via the medical, dental, and surgical arena. We must not flounder any longer. ...TSS View my complete profile

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Subject: BOVINE SPONGIFORM ENCEPHALOPATHY BSE TSE PRION UNITED STATE OF AMERICA UPDATE JULY 2016 Current Monthly Test Results

APHIS reports ongoing surveillance test totals monthly. Beginning in Fiscal Year 2016, the BSE ongoing surveillance program will sample approximately 25,000 animals each year. Under the program, USDA will continue to collect samples from a variety of sites and from the cattle populations where the disease is most likely to be detected.

Month

T E R R Y S . S I N G E L T A

J U L Y

Number of Tests

June 2016 May 2016 April 2016 R Y S R . March 2016 February 2016

1,876 2,329 1,973 2,564

January 2016 December 2015 November 2015 October 2015

3,095 2,102 1,891 775

2,901

Fiscal Year 2007-2015 Test Results For Fiscal Years 2007-2015, the BSE ongoing surveillance program had a target of testing approximately 40,000 animals per year. OIE Surveillance Points: design prevalence of 1 case per 100,000 U.S. adult cattle and 95% confidence; 300,000 OIE points over 7 years, or 42,857 OIE points on average per year are required. BSurvE Surveillance Points: design prevalence of 1 case per 1,000,000 U.S. adult cattle and 95% confidence; 3,000,000 points over 7 years, or 428,571 BSurvE points on average per year are required.

Samples Tested, OIE Points, and BSurvE Points by Fiscal Year Fiscal Year Number of OIE Points BSurvE Valid Tests Points FY 2015 FY 2014 FY 2013 FY 2012 FY 2011 FY 2010 FY 2009 FY 2008 FY 2007*

40,902 41,291 43,173 42,202 40,482 44,301 44,217 43,145 43,336

592,353 678,622 1,048,554 1,066,637 997,360 948,593 1,036,849 1,121,624 1,487,215

1,414,630 1,642,452 2,505,042 2,523,447 2,344,436 2,233,643 2,442,400 2,677,665 3,621,117

converted by Web2PDFConvert.com

* FY2007 - first complete year of BSE Ongoing Surveillance BSE Ongoing Surveillance Program Cumulative Totals Valid Tests From September 1, 2006 to June 28, 2016:

402,555 OIE Points From September 1, 2006 to June 28, 2016:

9,381,365 BSurvE Points From September 1, 2006 to June 28, 2016:

22,373,783 (see "BSE Ongoing Surveillance Plan" for information on OIE and BSurvE points) https://www.aphis.usda.gov/animal_health/animal_diseases/bse/download s/BSE_ongoing_surv_plan_final_71406.pdf https://www.aphis.usda.gov/aphis/ourfocus/animalhealth/animal-diseaseinformation/cattle-disease-information/sa_bse/ct_monthly_surv_test APHIS 91-55-082 https://www.aphis.usda.gov/animal_health/vet_accreditation/downloads/nv ap_ref_guide.pdf ADT and Reducing Response Time • 2006: Cow with BSE – 10 years old, no ID – Not linked with location, time frame USDA-APHIS National Veterinary Accreditation Program – DNA analysis unsuccessful – Took several weeks to investigate 37 farms – No definitive source – Incomplete data links Jul y 2014 Center for Food Security and Public Health ADT and Reducing Response Time (cont’d) • 2012: Cow with BSE – Sample collected at rendering facility – ICVIs issued by AVs li t d ID b USDA-APHIS National Veterinary Accreditation Program li s t e d ID num b ers, location, point in time – Found offspring, birth cohorts within days – Complete data links July 2014 Center for http://docplayer.net/8604112-1-27-2015-module-12-animal-diseasetraceability-supplemental-training-supplemental-training-supplementaltraining.html In 2015, the OIE determined that atypical BSE occurred spontaneously at a low rate in all cattle populations and would be excluded for BSE risk. https://www.aphis.usda.gov/aphis/ourfocus/animalhealth/animal-diseaseinformation/cattle-disease-information/sa_bse/ct_about_bse USDA Announces Preliminary Concurrence with World Animal Health Organization Risk Designations for Bovine Spongiform Encephalopathy in 16 Countries Published: Dec 4, 2015 Print December 4, 2015 -- The United States Department of Agriculture’s Animal and Plant Health Inspection Service (APHIS) is preliminarily concurring with the World Organization for Animal Health’s (OIE) bovine spongiform encephalopathy (BSE) risk designations for 16 countries. The OIE recognizes these regions as being of negligible risk for BSE. APHIS reviewed the information supporting the OIE’s risk designations for these regions and agrees with the OIE designations. The 16 countries are: Bulgaria, Cyprus, Czech Republic, Estonia, France,


India, Korea (Republic of), Hungary, Latvia, Liechtenstein, Luxembourg, Malta, Portugal, Romania, Slovakia, and Switzerland. The OIE recommendations regarding each of the above countries can be viewed online. APHIS considers all countries of the world to fall into in one of three BSE risk categories: negligible risk, controlled risk, or undetermined risk. Any region that is not classified by APHIS as presenting either negligible risk or controlled risk for BSE is considered to present an undetermined risk. Under the regulations, APHIS may classify a region for BSE in one of two ways. One way is for countries that have not received a risk classification from the World Organization for Animal Health (OIE) to request classification by APHIS. The other way is for APHIS to concur with the classification given to a country by the OIE. This notice is available for review online. APHIS will consider all comments received on or before February 2, 2016. After reviewing any comments we receive, we will announce our final determination regarding the BSE classification of these countries in the Federal Register. # USDA is an equal opportunity provider and employer. To file a complaint of discrimination, write: USDA, Office of the Assistant Secretary for Civil Rights, Office of Adjudication, 1400 Independence Ave., SW, Washington, DC 20250-9410 or call (866) 632-9992 (Toll-free Customer Service), (800) 877-8339 (Local or Federal relay), (866) 377-8642 (Relay voice users). https://www.aphis.usda.gov/aphis/newsroom/news/!ut/p/z0/hZDLTsNADE W_hUWXyJPQsG8riqj6QEKgMBvLSUwyTepJZ8yjf0YPbC7R7bOtQwWSrBCH64ldV5omPjV3uL2MF9lyyezvV8_3pnF82GzfNib 3OwL2ID9Y-Hl5seQh91q14IdSbtrJ28eyloxKvXcaHhgCTi36XmE4vGf5xT6eR0x_PZLsDWXpS_FEoaOxcxoSgOrgoULjMTCY U_Y_DlODX1jStLtiQcsq5yYoUsnxmpnuryBhc7LHh6FpJH4Kxt1UxtFffCMGudw!!/ BSE Cases Identified in the United States There have been 4 cases of BSE identified in the United States. The following information provides descriptions of these four cases: 2012 California collapsed On April 24, 2012, the USDA confirmed a BSE case in a dairy cow in California. This cow was tested as part of the USDA targeted BSE surveillance at rendering facilities in the United States. The cow was 10 years and 7 months old and was classified as having the Ltype BSE strain. For more information, see the USDA/APHIS notice, the SDA/APHIS Final Report[PDF – 179 KB] and the FDA Final Feed Investigation Summary. 2006 - Alabama collapsed On March 15, 2006, the USDA announced the confirmation of BSE in a cow in Alabama. The case was identified in a non-ambulatory (downer) cow on a farm in Alabama. The animal was euthanized by a local veterinarian and buried on the farm. The age of the cow was estimated by examination of the dentition as 10 years old. It had no ear tags or distinctive marks; the herd of origin could not be identified despite an intense investigation (see Alabama BSE Investigation, Final Epidemiology Report, May 2006[PDF - 105KB]). In August 2008, several ARS investigators reported that a rare, genetic abnormality that may persist within the cattle population "is considered to have caused" BSE in this atypical (H-type) BSE animal from Alabama. (See Identification of a Heritable Polymorphism in Bovine PRNP Associated with Genetic Transmissible Spongiform Encephalopathy: Evidence of Heritable BSE. Also see BSE Case Associated with Prion Protein Gene Mutation.) 2005 - Texas collapsed On June 24, 2005, the USDA announced receipt of final results from The Veterinary Laboratories Agency in Weybridge, England, confirming BSE in a cow that had conflicting test results in 2004. This cow was from Texas, died at approximately 12 years of age, and represented the first endemic case of BSE in the United States. (see Texas BSE Investigation, Final Epidemiology Report, August 2005[PDF 82KB]) 2003 - Washington State collapsed On December 23, 2003, the U.S. Department of Agriculture (USDA) announced a presumptive diagnosis of the first known case of BSE in the United States. It was in an adult Holstein cow from Washington State. This diagnosis was confirmed by an international reference laboratory in Weybridge, England, on December 25. Trace-back based on an ear-tag identification number and subsequent genetic testing confirmed that the BSE-infected cow was


imported into the United States from Canada in August 2001. Because the animal was non-ambulatory (a "downer cow") at slaughter, brain tissue samples were taken by USDA’s Animal and Plant Health Inspection Service as part of its targeted surveillance for BSE. However the animal’s condition was attributed to complications from calving. After the animal was examined by a USDA Food Safety and Inspection Service (FSIS) veterinary medical officer both before and after slaughter, the carcass was released for use as food for human consumption. During slaughter, the tissues considered to be at high risk for the transmission of the BSE agent were removed. On December 24, 2003, FSIS recalled beef from cattle slaughtered in the same plant on the same day as the BSE positive cow. (see Bovine Spongiform Encephalopathy in a Dairy Cow—Washington State, 2003(http://www.cdc.gov/mmwr/preview/mmwrhtml/mm5253a2.htm).) Preliminary Investigation Suggests BSE-Infected Cow in Washington State Was Likely Imported from Canada On December 23, 2003, the U.S. Department of Agriculture (USDA) announced a presumptive diagnosis of bovine spongiform encephalopathy (BSE, or "mad cow" disease) in an adult Holstein cow from Washington State. Samples were taken from the cow on December 9 as part of USDA's BSE surveillance program. The BSE diagnosis was made on December 22 and 23 by histopathology and immunohistochemical testing at the National Veterinary Services Laboratory, Ames, Iowa. The diagnosis was confirmed by an international reference laboratory in Weybridge, England, on December 25. Preliminary trace-back based on an ear-tag identification number suggests that the BSE-infected cow was imported into the United States from Canada in August 2001. USDA, in close cooperation with Canadian agricultural authorities, has launched an epidemiologic investigation to determine the source of the disease. Beef from the slaughtered cow had been processed for human consumption. On December 23, 2003, the Food Safety and Inspection Service (FSIS), USDA announced the recall of all beef from cattle slaughtered on December 9 at the involved slaughter plant. Strong evidence indicates that BSE has been transmitted to humans primarily in the United Kingdom, causing a variant form of CreutzfeldtJakob disease (vCJD)(http://www.cdc.gov/prions/vcjd/about.html). In the United Kingdom, where over 1 million cattle may have been infected with BSE, a substantial species barrier appears to protect humans from widespread illness. As of December 1, 2003, a total of 153 vCJD cases had been reported worldwide; of these, 143 cases had occurred in the United Kingdom. The risk to human health from BSE in the United States is extremely low. CDC monitors the trends and current incidence of CJD in the United States by analyzing death certificate information from U.S. multiple causeof-death data compiled by the National Center for Health Statistics. With the support of the Council of State and Territorial Epidemiologists, CDC conducts follow-up review of clinical and neuropathology records of CJD decedents younger than 55 years of age. In addition, during 1996-1997, in collaboration with the American Association of Neuropathologists (AANP), CDC established the National Prion Disease Pathology Surveillance Center at Case Western Reserve University in Cleveland, Ohio. This pathology center provides free, state-of-the-art diagnostic services to U.S. physicians. It also helps to monitor the possible occurrence of emerging forms of prion diseases, such as vCJD, in the United States. For more information about the center visit its website at: USDA About BSE FDA Bovine Spongiform Encephalopathy CDC Prion Diseases(http://www.cdc.gov/prions/index.html) NIH NINDS CreutzfeldtJakob Disease Information Page For more information about BSE in the United States, see the Animal and Plant Health Inspection Service, USDA BSE site. Related Links Prion Diseases(http://www.cdc.gov/prions/index.html) Chronic Wasting Disease (CWD) (http://www.cdc.gov/prions/cwd/index.html) Creutzfeldt-Jakob Disease (CJD)(http://www.cdc.gov/prions/cjd/index.html) Variant Creutzfeldt-Jakob Disease (vCJD)(http://www.cdc.gov/prions/vcjd/index.html) File Formats Help: How do I view different file formats (PDF, DOC, PPT, MPEG) on this site? Adobe PDF file Microsoft PowerPoint file Microsoft Word file Microsoft Excel file Audio/Video file Apple Quicktime file RealPlayer file Text file Zip Archive file SAS file ePub file RIS file Page last reviewed: February 10, 2015 Page last updated: February 10, 2015 Content source: Centers for Disease Control and Prevention National Center for Emerging and Zoonotic Infectious Diseases (NCEZID) Division of High-Consequence


Pathogens and Pathology (DHCPP) http://www.cdc.gov/prions/bse/case-us.html Comment from Terry Singeltary Sr. The is a Comment on the Animal and Plant Health Inspection Service (APHIS) Notice: Agency Information Collection Activities; Proposals, Submissions, and Approvals: Bovine Spongiform Encephalopathy; Importation of Animals and Animal Products For related information, Open Docket Folder -------------------------------------------------------------------------------Show agency attachment(s) AttachmentsView All (0) -------------------------------------------------------------------------------Comment View document:Docket No. APHIS-2014-0107 Bovine Spongiform Encephalopathy; Importation of Animals and Animal Products Singeltary Submission ; I believe that there is more risk to the world from Transmissible Spongiform Encephalopathy TSE prion aka mad cow type disease now, coming from the United States and all of North America, than there is risk coming to the USA and North America, from other Countries. I am NOT saying I dont think there is any risk for the BSE type TSE prion coming from other Countries, I am just saying that in 2015, why is the APHIS/USDA/FSIS/FDA still ignoring these present mad cow risk factors in North America like they are not here? North America has more strains of TSE prion disease, in more species (excluding zoo animals in the early BSE days, and excluding the Feline TSE and or Canine TSE, because they dont look, and yes, there has been documented evidence and scientific studies, and DEFRA Hound study, that shows the canine spongiform encephalopathy is very possible, if it has not already happened, just not documented), then any other Country in the world. Mink TME, Deer Elk cervid CWD (multiple strains), cBSE cattle, atypical L-type BSE cattle, atypical H-type BSE cattle, atyical HG type BSE cow (the only cow documented in the world to date with this strain), typical sheep goat Scrapie (multiple strains), and the atypical Nor98 Scrapie, which has been linked to sporadic CJD, Nor-98 atypical Scrapie has spread from coast to coast. sporadic CJD on the rise, with different strains mounting, victims becoming younger, with the latest nvCJD human mad cow case being documented in Texas again, this case, NOT LINKED TO EUROPEAN TRAVEL CDC. typical BSE can propagate as nvCJD and or sporadic CJD (Collinge et al), and sporadic CJD has now been linked to atypical BSE, Scrapie and atypical Scrapie, and scientist are very concerned with CWD TSE prion in the Cervid populations. in my opinion, the BSE MRR policy, which overtook the BSE GBR risk assessments for each country, and then made BSE confirmed countries legal to trade mad cow disease, which was all brought forth AFTER that fateful day December 23, 2003, when the USA lost its gold card i.e. BSE FREE status, thats the day it all started. once the BSE MRR policy was shoved down every countries throat by USDA inc and the OIE, then the legal trading of Scrapie was validated to be a legal trading commodity, also shoved through by the USDA inc and the OIE, the world then lost 30 years of attempted eradication of the BSE TSE prion disease typical and atypical strains, and the BSE TSE Prion aka mad cow type disease was thus made a legal trading commodity, like it or not. its all about money now folks, trade, to hell with human health with a slow incubating disease, that is 100% fatal once clinical, and forget the fact of exposure, sub-clinical infection, and friendly fire there from i.e. iatrogenic TSE prion disease, the pass it forward mode of the TSE PRION aka mad cow type disease. its all going to be sporadic CJD or sporadic ffi, or sporadic gss, or now the infamous VPSPr. ...problem solved $$$ the USDA/APHIS/FSIS/FDA triple mad cow BSE firewall, well, that was nothing but ink on paper. for this very reason I believe the BSE MRR policy is a total failure, and that this policy should be immediately withdrawn, and set back in place the BSE GBR Risk Assessments, with the BSE GBR risk assessments set up to monitor all TSE PRION disease in all species of animals, and that the BSE GBR risk assessments be made stronger than before. lets start with the recent notice that beef from Ireland will be coming to America.


Ireland confirmed around 1655 cases of mad cow disease. with the highest year confirming about 333 cases in 2002, with numbers of BSE confirmed cases dropping from that point on, to a documentation of 1 confirmed case in 2013, to date. a drastic decrease in the feeding of cows to cows i.e. the ruminant mad cow feed ban, and the enforcement of that ban, has drastically reduced the number of BSE cases in Europe, minus a few BABs or BARBs. a far cry from the USDA FDA triple BSE firewall, which was nothing more than ink on paper, where in 2007, in one week recall alone, some 10 MILLION POUNDS OF BANNED POTENTIAL MAD COW FEED WENT OUT INTO COMMERCE IN THE USA. this is 10 years post feed ban. in my honest opinion, due to the blatant cover up of BSE TSE prion aka mad cow disease in the USA, we still have no clue as to the true number of cases of BSE mad cow disease in the USA or North America as a whole. ...just saying. Number of reported cases of bovine spongiform encephalopathy (BSE) in farmed cattle worldwide* (excluding the United Kingdom) Country/Year snip...please see attached pdf file, with references of breaches in the USA triple BSE mad cow firewalls, and recent science on the TSE prion disease. ...TSS No documents available. AttachmentsView All (1) Docket No. APHIS-2014-0107 Bovine Spongiform Encephalopathy; Importation of Animals and Animal Products Singeltary Submission View Attachment: https://www.regulations.gov/document?D=APHIS-2014-0107-0003 Terry S. Singeltary Sr. submission ; http://www.regulations.gov/#!documentDetail;D=FDA-2014-D-1180-0003 Docket No. APHIS-2014-0107 Bovine Spongiform Encephalopathy; Importation of Animals and Animal Products Singeltary Submission Posted: 12/30/2014ID: APHIS-2014-0107-0001 http://www.regulations.gov/#!documentDetail;D=APHIS-2014-0107-0003 Notice: Environmental Impact Statements; Availability, etc.: Animal Carcass Management Document ID: APHIS-2013-0044-0001 Docket ID: APHIS-2013-0044 Comment ID: APHIS-2013-0044-0002 http://www.noticeandcomment.com/APHIS-2013-0044-0002-fcod365217.aspx (APHIS) Notice: Agency Information Collection Activities; Proposals, Submissions, and Approvals: Chronic Wasting Disease Herd Certification Program Agency Information Collection Activities; Proposals, Submissions, and Approvals: Chronic Wasting Disease Herd Certification Program (Document ID APHIS-2011-0032-0001) http://www.regulations.gov/#!documentDetail;D=APHIS-2011-0032-0002 Owens, Julie From: Terry S. Singeltary Sr. [[email protected]] 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 http://www.fsis.usda.gov/OPPDE/Comments/2006-0011/2006-0011-1.pdf FSIS, USDA, REPLY TO SINGELTARY http://www.fsis.usda.gov/PDF/BSE_Risk_Assess_Response_Public_Com ments.pdf From:Terry S. Singeltary Sr. [[email protected]] Sent:Thursday, September 08, 2005 6:17 PM To:[email protected] Subject: [Docket No. 03-025IFA] FSIS Prohibition of the Use of Specified


Risk Materials for Human Food and Requirements for the Disposition of Non-Ambulatory Disabled Cattle http://www.fsis.usda.gov/OPPDE/Comments/03-025IFA/03-025IFA-2.pdf APHIS-2006-0118-0096 CWD http://www.regulations.gov/#!documentDetail;D=APHIS-2006-0118-0100 DOCKET-- 03D-0186 -- FDA Issues Draft Guidance on Use of Material From Deer and Elk in Animal Feed; Availability Date: Fri, 16 May 2003 11:47:37 0500 EMC 1 Terry S. Singeltary Sr. Vol #: 1 http://www.fda.gov/ohrms/dockets/dailys/03/Jun03/060903/060903.htm http://www.fda.gov/ohrms/dockets/dailys/03/oct03/100203/100203.htm PLEASE SEE FULL TEXT SUBMISSION ; http://madcowfeed.blogspot.com/2008/07/docket-03d-0186-fda-issuesdraft.html *** 2001 Terry S. Singeltary Sr. comment submission *** http://www.fda.gov/ohrms/dockets/ac/01/slides/3681s2_09.pdf when USDA inc. contracts out for someone to pick up suspect BSE mad cow cattle heads for testing, you would hope they would be from suspect BSE mad cow cattle. NOT WITH THE USDA INC., they hired someone to pick up HEALTHY CATTLE THEY KNEW DID NOT HAVE MAD COW DISEASE BSE TO BE TESTED FOR MAD COW DISEASE BSE. Subject: USDA OIG SEMIANNUAL REPORT TO CONGRESS FY 2007 1st Half (bogus BSE sampling FROM HEALTHY USDA CATTLE) Date: June 21, 2007 at 2:49 pm PST Owner and Corporation Plead Guilty to Defrauding Bovine Spongiform Encephalopathy (BSE) Surveillance Program An Arizona meat processing company and its owner pled guilty in February 2007 to charges of theft of Government funds, mail fraud, and wire fraud. The owner and his company defrauded the BSE Surveillance Program when they falsified BSE Surveillance Data Collection Forms and then submitted payment requests to USDA for the services. In addition to the targeted sample population (those cattle that were more than 30 months old or had other risk factors for BSE), the owner submitted to USDA, or caused to be submitted, BSE obex (brain stem) samples from healthy USDA-inspected cattle. As a result, the owner fraudulently received approximately $390,000. Sentencing is scheduled for May 2007. snip... Topics that will be covered in ongoing or planned reviews under Goal 1 include: soundness of BSE maintenance sampling (APHIS), implementation of Performance-Based Inspection System enhancements for specified risk material (SRM) violations and improved inspection controls over SRMs (FSIS and APHIS), snip... The findings and recommendations from these efforts will be covered in future semiannual reports as the relevant audits and investigations are completed. 4 USDA OIG SEMIANNUAL REPORT TO CONGRESS FY 2007 1st Half http://www.usda.gov/oig/webdocs/sarc070619.pdf 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 $$$ From: Terry S. Singeltary Sr. (216-119-138-129.ipset18.wt.net)


Subject: Emergency Operations...BSE Red Book Date: March 13, 2000 at 1:30 pm PST BSE Red Book 2.1-35 7.0 Emergency Operations The section below would be implemented only after a first case of BSE is confirmed in the United States. 7.1 READEO Activation Prelimanary Notification The director of NVSL is responsible for immediately notifying the APHIS, Veterinary Services (VS) deputy administrator when tests suggest a presumptive diagnosis of BSE. Once NVSL has made a presumptive diagnosis of BSE, APHIS and FSIS field activities will also be initiated. APHIS will receive notification (either confirming or not confirming NVSL's diagnosis) from the United Kingdom anywhere between 24 and 96 hours http://madcowtesting.blogspot.com/2009/07/us-emergency-bovinespongiform.html IT TOOK 7 MONTHS AND AN ACT OF CONGRESS TO GET THIS 2ND TEXAS BSE MAD COW CONFIRMED ! now, someone please tell me why I should expect anything different with Chronic Wasting Disease CWD TSE Prion testing in Texas $$$ the good old boy system is still alive and well in Texas i.e. shoot, shovel, and shut the hell up i.e. SSS POLICY... Tuesday, April 24, 2012 MAD COW DISEASE USA 4TH CASE DOCUMENTED ATYPICAL BSE CALIFORNIA http://transmissiblespongiformencephalopathy.blogspot.com/2012/04/madcow-disease-usa-4th-case-documented.html Wednesday, April 25, 2012 4th MAD COW DISEASE U.S.A. CALIFORNIA ATYPICAL L-TYPE BSE 2012 http://transmissiblespongiformencephalopathy.blogspot.com/2012/04/4thmad-cow-disease-usa-california.html Qualitative Analysis of BSE Risk Factors in the United States February 13, 2000 at 3:37 pm PST (BSE red book) http://bseusa.blogspot.com/2008/08/qualitative-analysis-of-bse-risk.html Sunday, May 27, 2012 CANADA PLANS TO IMPRISON ANYONE SPEAKING ABOUT MAD COW or ANY OTHER DISEASE OUTBREAK, CENSORSHIP IS A TERRIBLE THING http://madcowusda.blogspot.com/2012/05/canada-plans-to-imprisonanyone.html Thursday, April 26, 2012 FDA Statement on USDA Announcement of Positive BSE Test Result For Immediate Release: April 26, 2012 http://transmissiblespongiformencephalopathy.blogspot.com/2012/04/fdastatement-on-usda-announcement-of.html 2016 Wednesday, May 25, 2016 USDA APHIS National Scrapie TSE Prion Eradication Program April 2016 Monthly Report Prion 2016 Tokyo Update http://scrapie-usa.blogspot.com/2016/05/usda-aphis-national-scrapie-tseprion.html


Friday, April 22, 2016 Texas Scrapie Confirmed in a Hartley County Sheep where CWD was detected in a Mule Deer http://scrapie-usa.blogspot.com/2016/04/texas-scrapie-confirmed-inhartley.html Sunday, July 10, 2016 Primary transmission of CWD versus scrapie prions from small ruminants to ovine and cervid PrP transgenic mice http://chronic-wasting-disease.blogspot.com/2016/07/primarytransmission-of-cwd-versus.html Tuesday, July 12, 2016 Chronic Wasting Disease CWD, Scrapie, Bovine Spongiform Encephalopathy BSE, TSE, Prion Zoonosis Science History see history of NIH may destroy human brain collection http://transmissiblespongiformencephalopathy.blogspot.com/2016/07/chro nic-wasting-disease-cwd-scrapie.html Monday, May 09, 2016 A comparison of classical and H-type bovine spongiform encephalopathy associated with E211K prion protein polymorphism in wild type and EK211 cattle following intracranial inoculation http://bse-atypical.blogspot.com/2016/05/a-comparison-of-classical-andh-type.html *** Docket No. APHIS-2007-0127 Scrapie in Sheep and Goats Terry Singeltary Sr. Submission *** Docket No. APHIS-2007-0127 Scrapie in Sheep and Goats SUMMARY: We are reopening the comment period for our proposed rule that would revise completely the scrapie regulations, which concern the risk groups and categories established for individual animals and for flocks, the use of genetic testing as a means of assigning risk levels to animals, movement restrictions for animals found to be genetically less susceptible or resistant to scrapie, and recordkeeping requirements. This action will allow interested persons additional time to prepare and submit comments.DATES: The comment period for the proposed rule published on September 10, 2015 (80 FR 54660-54692) is reopened. We will consider all comments that we receive on or before December 9, 2015. ... http://www.regulations.gov/#!documentDetail;D=APHIS-2007-0127-0001 http://www.gpo.gov/fdsys/pkg/FR-2015-11-16/html/2015-29179.htm http://www.regulations.gov/#!docketDetail;D=APHIS-2007-0127 Comment from Terry Singeltary This is a Comment on the Animal and Plant Health Inspection Service (APHIS) Proposed Rule: Scrapie in Sheep and Goats For related information, Open Docket Folder Docket folder icon -------------------------------------------------------------------------------Show agency attachment(s) AttachmentsView All (0) -------------------------------------------------------------------------------Comment View document:Indeed, much science has changed about the Scrapie TSE prion, including more science linking Scrapie to humans. sadly, politics, industry, and trade, have not changed, and those usually trump sound science, as is the case with all Transmissible Spongiform Encephalopathy TSE Prion disease in livestock producing animals and the OIE. we can look no further at the legal trading of the Scrapie TSE prion both typical and atypical of all strains, and CWD all stains. With as much science of old, and now more new science to back this up, Scrapie of all types i.e. atypical and typical, BSE all strains, and CWD all strains, should be regulated in trade as BSE TSE PRION. In fact, I urge APHIS et


al and the OIE, and all trading partners to take heed to the latest science on the TSE prion disease, all of them, and seriously reconsider the blatant disregards for human and animal health, all in the name of trade, with the continued relaxing of TSE Prion trade regulations through the 'NEGLIGIBLE BSE RISK' PROGRAM, which was set up to fail in the first place. If the world does not go back to the 'BSE RISK ASSESSMENTS', enhance, and or change that assessment process to include all TSE prion disease, i.e. 'TSE RISK ASSESSMENT', if we do not do this and if we continue this farce with OIE and the USDA et al, and the 'NEGLIGIBLE BSE RISK' PROGRAM, we will never eradicate the TSE prion aka mad cow type disease, they will continue to mutate and spread among species of human and animal origin, and they will continue to kill. ... please see ; O.05: Transmission of prions to primates after extended silent incubation periods: Implications for BSE and scrapie risk assessment in human populations Emmanuel Comoy, Jacqueline Mikol, Valerie Durand, Sophie Luccantoni, Evelyne Correia, Nathalie Lescoutra, Capucine Dehen, and Jean-Philippe Deslys Atomic Energy Commission; Fontenay-aux-Roses, France Prion diseases (PD) are the unique neurodegenerative proteinopathies reputed to be transmissible under field conditions since decades. The transmission of Bovine Spongiform Encephalopathy (BSE) to humans evidenced that an animal PD might be zoonotic under appropriate conditions. Contrarily, in the absence of obvious (epidemiological or experimental) elements supporting a transmission or genetic predispositions, PD, like the other proteinopathies, are reputed to occur spontaneously (atpical animal prion strains, sporadic CJD summing 80% of human prion cases). Non-human primate models provided the first evidences supporting the transmissibiity of human prion strains and the zoonotic potential of BSE. Among them, cynomolgus macaques brought major information for BSE risk assessment for human health (Chen, 2014), according to their phylogenetic proximity to humans and extended lifetime. We used this model to assess the zoonotic potential of other animal PD from bovine, ovine and cervid origins even after very long silent incubation periods. *** We recently observed the direct transmission of a natural classical scrapie isolate to macaque after a 10-year silent incubation period, ***with features similar to some reported for human cases of sporadic CJD, albeit requiring fourfold longe incubation than BSE. Scrapie, as recently evoked in humanized mice (Cassard, 2014), ***is the third potentially zoonotic PD (with BSE and L-type BSE), ***thus questioning the origin of human sporadic cases. We will present an updated panorama of our different transmission studies and discuss the implications of such extended incubation periods on risk assessment of animal PD for human health. =============== ***thus questioning the origin of human sporadic cases*** =============== https://prion2015.files.wordpress.com/2015/05/prion2015abstracts.pdf ***This information will have a scientific impact since it is the first study that demonstrates the transmission of scrapie to a non-human primate with a close genetic relationship to humans. This information is especially useful to regulatory officials and those involved with risk assessment of the potential transmission of animal prion diseases to humans. ***This observation strengthens the questioning of the harmlessness of scrapie to humans, at a time when protective measures for human and animal health are being dismantled and reduced as c-BSE is considered controlled and being eradicated. Our results underscore the importance of precautionary and protective measures and the necessity for long-term experimental transmission studies to assess the zoonotic potential of other animal prion strains. http://www.ars.usda.gov/research/publications/publications.htm? SEQ_NO_115=313160 please see file attachment for full submission and recent science and my deep concerns on the TSE Prion disease... No documents available.


AttachmentsView All (1) scrapie-usa-blogspot-com View Attachment: http://www.regulations.gov/#!documentDetail;D=APHIS-2007-0127-0032 Saturday, July 16, 2016 Importation of Sheep, Goats, and Certain Other Ruminants [Docket No. APHIS-2009-0095]RIN 0579-AD10 WITH great disgust and concern, I report to you that the OIE, USDA, APHIS, are working to further legalize the trading of Transmissible Spongiform Encephalopathy TSE Pion disease around the globe. THIS is absolutely insane. it’s USDA INC. http://scrapie-usa.blogspot.com/2016/07/importation-of-sheep-goats-andcertain.html USDA BSE TSE PRION SURVEILLANCE, FEED, TESTING, SRM FIREWALLS...LMAO! THE USDA FDA TRIPLE MAD COW DISEASE FIREWALL, WERE NOTHING MORE THAN INK ON PAPER ! infamous august 4, 1997 BSE TSE prion mad cow feed ban, part of usda fda et al TRIPLE MAD COW FIREWALL, 10 YEARS AFTER ; 10,000,000+ LBS. of PROHIBITED BANNED MAD COW FEED I.E. BLOOD LACED MBM IN COMMERCE USA 2007 Date: March 21, 2007 at 2:27 pm PST RECALLS AND FIELD CORRECTIONS: VETERINARY MEDICINES -- CLASS II PRODUCT Bulk cattle feed made with recalled Darling's 85% Blood Meal, Flash Dried, Recall # V-024-2007 CODE Cattle feed delivered between 01/12/2007 and 01/26/2007 RECALLING FIRM/MANUFACTURER Pfeiffer, Arno, Inc, Greenbush, WI. by conversation on February 5, 2007. Firm initiated recall is ongoing. REASON Blood meal used to make cattle feed was recalled because it was cross- contaminated with prohibited bovine meat and bone meal that had been manufactured on common equipment and labeling did not bear cautionary BSE statement. VOLUME OF PRODUCT IN COMMERCE 42,090 lbs. DISTRIBUTION WI ___________________________________ PRODUCT Custom dairy premix products: MNM ALL PURPOSE Pellet, HILLSIDE/CDL Prot- Buffer Meal, LEE, M.-CLOSE UP PX Pellet, HIGH DESERT/ GHC LACT Meal, TATARKA, M CUST PROT Meal, SUNRIDGE/CDL PROTEIN Blend, LOURENZO, K PVM DAIRY Meal, DOUBLE B DAIRY/GHC LAC Mineral, WEST PIONT/GHC CLOSEUP Mineral, WEST POINT/GHC LACT Meal, JENKS, J/COMPASS PROTEIN Meal, COPPINI - 8# SPECIAL DAIRY Mix, GULICK, L-LACT Meal (Bulk), TRIPLE J - PROTEIN/LACTATION, ROCK CREEK/GHC MILK Mineral, BETTENCOURT/GHC S.SIDE MK-MN, BETTENCOURT #1/GHC MILK MINR, V&C DAIRY/GHC LACT Meal, VEENSTRA, F/GHC LACT Meal, SMUTNY, A- BYPASS ML W/SMARTA, Recall # V-025-2007 CODE The firm does not utilize a code - only shipping documentation with commodity and weights identified. RECALLING FIRM/MANUFACTURER Rangen, Inc, Buhl, ID, by letters on February 13 and 14, 2007. Firm initiated recall is complete. REASON Products manufactured from bulk feed containing blood meal that was cross contaminated with prohibited meat and bone meal and the labeling did not bear cautionary BSE statement. VOLUME OF PRODUCT IN COMMERCE 9,997,976 lbs. DISTRIBUTION ID and NV END OF ENFORCEMENT REPORT FOR MARCH 21, 2007 http://www.fda.gov/Safety/Recalls/EnforcementReports/2007/ucm120446.h tm 16 years post mad cow feed ban August 1997 2013 Sunday, December 15, 2013 FDA PART 589 -- SUBSTANCES PROHIBITED FROM USE IN ANIMAL


FOOD OR FEED VIOLATIONS OFFICIAL ACTION INDICATED OIA UPDATE DECEMBER 2013 UPDATE http://madcowusda.blogspot.com/2013/12/fda-part-589-substancesprohibited-from.html 17 years post mad cow feed ban August 1997 Tuesday, December 23, 2014 FDA PART 589 -- SUBSTANCES PROHIBITED FROM USE IN ANIMAL FOOD OR FEED VIOLATIONS OFFICIAL ACTION INDICATED OAI UPDATE DECEMBER 2014 BSE TSE PRION http://madcowusda.blogspot.com/2014/12/fda-part-589-substancesprohibited-from.html *** Monday, October 26, 2015 *** *** FDA PART 589 -- SUBSTANCES PROHIBITED FROM USE IN ANIMAL FOOD OR FEED VIOLATIONS OFFICIAL ACTION INDICATED OIA UPDATE October 2015 *** http://madcowusda.blogspot.com/2015/10/fda-part-589-substancesprohibited-from.html Thursday, July 24, 2014 *** Protocol for further laboratory investigations into the distribution of infectivity of Atypical BSE SCIENTIFIC REPORT OF EFSA New protocol for Atypical BSE investigations http://bse-atypical.blogspot.com/2014/07/protocol-for-furtherlaboratory.html *** Singeltary reply ; Molecular, Biochemical and Genetic Characteristics of BSE in Canada Singeltary reply ; http://www.plosone.org/annotation/listThread.action;jsessionid=635CE909 4E0EA15D5362B7D7B809448C?root=7143 Saturday, January 31, 2015 European red deer (Cervus elaphus elaphus) are susceptible to Bovine Spongiform Encephalopathy BSE by Oral Alimentary route http://transmissiblespongiformencephalopathy.blogspot.com/2015/01/euro pean-red-deer-cervus-elaphus.html I strenuously once again urge the FDA and its industry constituents, to make it MANDATORY that all ruminant feed be banned to all ruminants, and this should include all cervids as soon as possible for the following reasons... ====== In the USA, under the Food and Drug Administrations BSE Feed Regulation (21 CFR 589.2000) most material (exceptions include milk, tallow, and gelatin) from deer and elk is prohibited for use in feed for ruminant animals. With regards to feed for non-ruminant animals, under FDA law, CWD positive deer may not be used for any animal feed or feed ingredients. For elk and deer considered at high risk for CWD, the FDA recommends that these animals do not enter the animal feed system. ***However, this recommendation is guidance and not a requirement by law. ====== 31 Jan 2015 at 20:14 GMT *** Ruminant feed ban for cervids in the United States? *** 31 Jan 2015 at 20:14 GMT see Singeltary comment ; http://www.plosone.org/annotation/listThread.action?root=85351 Docket No. FDA-2003-D-0432 (formerly 03D-0186) Use of Material from Deer and Elk in Animal Feed Singeltary Submission


https://www.regulations.gov/#!documentDetail;D=FDA-2003-D-0432-0011 http://www.plosone.org/annotation/listThread.action?root=85351 http://journals.plos.org/plosone/article/comment? id=info:doi/10.1371/annotation/09676b86-bbc2-4c69-9032-c319f13a7ad0 see BSE TSE SRM breaches being served up to humans as appetizers... Monday, June 20, 2016 Specified Risk Materials SRMs BSE TSE Prion Program http://specifiedriskmaterial.blogspot.com/2016/06/specified-risk-materialssrms-bse-tse.html 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 Maximizing profits is all that is going on now, thanks to the OIE BSE MRR policy, the legal trading of all strains of TSE prion disease globally. ...Terry S. Singeltary Sr. atypical BSE spontaneous sporadic ??? Saturday, May 26, 2012 Are USDA assurances on mad cow case 'gross oversimplification'? SNIP... *** What irks many scientists is the USDA’s April 25 statement that the rare disease is “not generally associated with an animal consuming infected feed.” *** The USDA’s conclusion is a “gross oversimplification,” said Dr. Paul Brown, one of the world’s experts on this type of disease who retired recently from the National Institutes of Health. *** "(The agency) has no foundation on which to base that statement.” *** “We can’t say it’s not feed related,” agreed Dr. Linda Detwiler, an official with the USDA during the Clinton Administration now at Mississippi State. *** In the May 1 email to me, USDA’s Cole backed off a bit. “No one knows the origins of atypical cases of BSE,” she said *** The argument about feed is critical because if feed is the cause, not a spontaneous mutation, the California cow could be part of a larger outbreak. SNIP... http://bseusa.blogspot.com/2012/05/are-usda-assurances-on-mad-cowcase.html Evidence That Transmissible Mink Encephalopathy Results from Feeding Infected Cattle 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://collections.europarchive.org/tna/20090505194948/http://bseinquiry.g ov.uk/files/mb/m09/tab05.pdf


http://collections.europarchive.org/tna/20090505194948/http://bseinquiry.g ov.uk/files/mb/m09a/tab01.pdf http://collections.europarchive.org/tna/20090505194948/http://bseinquiry.g ov.uk/files/yb/1987/06/10004001.pdf In Confidence - Perceptions of unconventional slow virus diseases of animals in the USA - APRIL-MAY 1989 - G A H Wells 3. Prof. A. Robertson gave a brief account of BSE. The US approach was to accord it a very low profile indeed. Dr. A Thiermann showed the picture in the ''Independent'' with cattle being incinerated and thought this was a fanatical incident to be avoided in the US at all costs. ... http://web.archive.org/web/20060307063531/http://www.bseinquiry.gov.uk/f iles/mb/m11b/tab01.pdf ”The occurrence of CWD must be viewed against the contest of the locations in which it occurred. It was an incidental and unwelcome complication of the respective wildlife research programmes. Despite it’s subsequent recognition as a new disease of cervids, therefore justifying direct investigation, no specific research funding was forthcoming. The USDA veiwed it as a wildlife problem and consequently not their province!” ...page 26. http://collections.europarchive.org/tna/20080102193705/http://www.bseinq uiry.gov.uk/files/mb/m11b/tab01.pdf *** Spraker suggested an interesting explanation for the occurrence of CWD. The deer pens at the Foot Hills Campus were built some 30-40 years ago by a Dr. Bob Davis. At or abut that time, allegedly, some scrapie work was conducted at this site. When deer were introduced to the pens they occupied ground that had previously been occupied by sheep. http://collections.europarchive.org/tna/20080102193705/http://www.bseinq uiry.gov.uk/files/mb/m11b/tab01.pdf Atypical BSE (BASE) Transmitted from Asymptomatic Aging Cattle to a Primate Emmanuel E. Comoy1*, Cristina Casalone2, Nathalie LescoutraEtchegaray1, Gianluigi Zanusso3, Sophie Freire1, Dominique Marcé1, Frédéric Auvré1, Marie-Magdeleine Ruchoux1, Sergio Ferrari3, Salvatore Monaco3, Nicole Salès4, Maria Caramelli2, Philippe Leboulch1,5, Paul Brown1, Corinne I. Lasmézas4, Jean-Philippe Deslys1 1 Institute of Emerging Diseases and Innovative Therapies, CEA, Fontenay-aux-Roses, France, 2 Istituto Zooprofilattico Sperimentale del Piemonte, Turin, Italy, 3 Policlinico G.B. Rossi, Verona, Italy, 4 Scripps Florida, Jupiter, Florida, United States of America, 5 Genetics Division, Brigham & Women's Hospital, Harvard Medical School, Boston, Massachusetts, United States of America Abstract Top Background Human variant Creutzfeldt-Jakob Disease (vCJD) results from foodborne transmission of prions from slaughtered cattle with classical Bovine Spongiform Encephalopathy (cBSE). Atypical forms of BSE, which remain mostly asymptomatic in aging cattle, were recently identified at slaughterhouses throughout Europe and North America, raising a question about human susceptibility to these new prion strains. Methodology/Principal Findings Brain homogenates from cattle with classical BSE and atypical (BASE) infections were inoculated intracerebrally into cynomolgus monkeys (Macacca fascicularis), a non-human primate model previously demonstrated to be susceptible to the original strain of cBSE. The resulting diseases were compared in terms of clinical signs, histology and biochemistry of the abnormal prion protein (PrPres). The single monkey infected with BASE had a shorter survival, and a different clinical evolution, histopathology, and prion protein (PrPres) pattern than was observed for either classical BSE or vCJD-inoculated animals. Also, the biochemical signature of PrPres in the BASE-inoculated animal was found to have a higher proteinase K sensitivity of the octa-repeat region. We found the same biochemical signature in three of four human patients with sporadic CJD and an MM type 2 PrP genotype who lived in the same country as the infected bovine.


Conclusion/Significance Our results point to a possibly higher degree of pathogenicity of BASE than classical BSE in primates and also raise a question about a possible link to one uncommon subset of cases of apparently sporadic CJD. Thus, despite the waning epidemic of classical BSE, the occurrence of atypical strains should temper the urge to relax measures currently in place to protect public health from accidental contamination by BSE-contaminated products. Citation: Comoy EE, Casalone C, Lescoutra-Etchegaray N, Zanusso G, Freire S, et al. (2008) Atypical BSE (BASE) Transmitted from Asymptomatic Aging Cattle to a Primate. PLoS ONE 3(8): e3017. doi:10.1371/journal.pone.0003017 Editor: Neil Mabbott, University of Edinburgh, United Kingdom Received: April 24, 2008; Accepted: August 1, 2008; Published: August 20, 2008 Copyright: © 2008 Comoy 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. Funding: This work has been supported by the Network of Excellence NeuroPrion. Competing interests: CEA owns a patent covering the BSE diagnostic tests commercialized by the company Bio-Rad. * E-mail: [email protected] http://www.plosone.org/article/info:doi%2F10.1371%2Fjournal.pone.00030 17 atypical Bovine Amyloidotic Spongiform Encephalopathy Bovine Spongiform Encephalopathy 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. 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 diseaseassociated 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... 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/ Monday, May 23, 2011 Atypical Prion Diseases in Humans and Animals 2011 Top Curr Chem (2011) DOI: 10.1007/128_2011_161 # Springer-Verlag Berlin Heidelberg 2011 Michael A. Tranulis, Sylvie L. Benestad, Thierry Baron, and Hans Kretzschmar Abstract Although prion diseases, such as Creutzfeldt-Jakob disease (CJD) in humans and scrapie in sheep, have long been recognized, our understanding of their epidemiology and pathogenesis is still in its early stages. Progress is hampered by the lengthy incubation periods and the lack of effective ways of monitoring and characterizing these agents. Protease-resistant conformers of the prion protein (PrP), known as the "scrapie form" (PrPSc), are used as disease markers, and for taxonomic purposes, in correlation with clinical, pathological, and genetic data. In humans, prion diseases can arise sporadically (sCJD) or genetically (gCJD and others), caused by mutations in the PrP-gene (PRNP), or as a foodborne infection, with the agent of bovine spongiform encephalopathy (BSE) causing variant CJD (vCJD). Person-to-person spread of human prion disease has only been known to occur following cannibalism (kuru disease in Papua New Guinea) or through medical or surgical treatment (iatrogenic CJD, iCJD). In contrast, scrapie in small ruminants and chronic wasting disease (CWD) in cervids behave as infectious diseases within these species. Recently, however, so-called atypical forms of prion diseases have been discovered in sheep (atypical/Nor98 scrapie) and in cattle, BSE-H and BSE-L. These maladies resemble sporadic or genetic human prion diseases and might be their animal equivalents. This hypothesis also raises the significant public health question of possible epidemiological links between these diseases and their counterparts in humans. M.A. Tranulis (*) Norwegian School of Veterinary Science, Oslo, Norway e-mail: [email protected] S.L. Benestad Norwegian Veterinary Institute, Oslo, Norway T. Baron


Agence Nationale de Sećurite´ Sanitaire, ANSES, Lyon, France H. Kretzschmar Ludwig-Maximilians University of Munich, Munich, Germany Keywords Animal Atypical Atypical/Nor98 scrapie BSE-H BSE-L Human Prion disease Prion strain Prion type http://resources.metapress.com/pdf-preview.axd? code=f433r34h34ugg617&size=largest 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. http://www.prionetcanada.ca/detail.aspx? menu=5&dt=293380&app=93&cat1=387&tp=20&lk=no&cat2 L-BSE, TME, AND SPORADIC CJD in North America Moreover, transmission experiments to non-human primates suggest that some TSE agents in addition to Classical BSE prions in cattle (namely Ltype 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 Transmissibility of BSE-L and Cattle-Adapted TME Prion Strain to Cynomolgus Macaque 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: [email protected] 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. Cattleadapted 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 BSEL, 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, bovinepassaged 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 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/trans missibility-of-bse-l-and-cattle.html Thursday, April 26, 2012 Update from USDA Regarding a Detection of Bovine Spongiform Encephalopathy (BSE) in the United States WASHINGTON bulletin at 04/26/2012 10:11 PM EDT http://transmissiblespongiformencephalopathy.blogspot.com/2012/04/upda te-from-usda-regarding-detection-of.html Saturday, August 4, 2012 Final Feed Investigation Summary - California BSE Case - July 2012 http://transmissiblespongiformencephalopathy.blogspot.com/2012/08/finalfeed-investigation-summary.html SUMMARY REPORT CALIFORNIA BOVINE SPONGIFORM ENCEPHALOPATHY CASE INVESTIGATION JULY 2012 Summary Report BSE 2012 Executive Summary http://transmissiblespongiformencephalopathy.blogspot.com/2012/08/sum mary-report-california-bovine.html Saturday, August 4, 2012 Update from APHIS Regarding Release of the Final Report on the BSE Epidemiological Investigation http://transmissiblespongiformencephalopathy.blogspot.com/2012/08/upda te-from-aphis-regarding-release-of.html Information released on 2 February 2005 Summary of information requested What statistics are available on cattle less than 30 months of age found to have BSE? Information released VLA has recorded approximately 100 cases of BSE in cattle of 30 months of age or under during the entire period of the BSE epidemic (1986 - 2005).


The figure is approximate as for 51 of these the age is only estimated. This is because farmers did not have accurate documentation to confirm birth date. This was not a requirement at the time. We can confirm that of the 100 cases, 49 were under 30 months of age, of these the youngest case was 20 months old. http://vla.defra.gov.uk/vla/vla_ati_020205.htm http://bseyoungestage.blogspot.com/2008/05/bse-youngest-age-statisticsunder-30.html PRION 2016 CONFERENCE TOKYO Saturday, April 23, 2016 SCRAPIE WS-01: Prion diseases in animals and zoonotic potential 2016 Prion. 10:S15-S21. 2016 ISSN: 1933-6896 printl 1933-690X online Taylor & Francis Prion 2016 Animal Prion Disease Workshop Abstracts WS-01: Prion diseases in animals and zoonotic potential Juan Maria Torres a, Olivier Andreoletti b, J uan-Carlos Espinosa a. Vincent Beringue c. Patricia Aguilar a, Natalia Fernandez-Borges a. and Alba Marin-Moreno a "Centro de Investigacion en Sanidad Animal ( CISA-INIA ). Valdeolmos, Madrid. Spain; b UMR INRA -ENVT 1225 Interactions Holes Agents Pathogenes. ENVT. Toulouse. France: "UR892. Virologie lmmunologie MolécuIaires, Jouy-en-Josas. France Dietary exposure to bovine spongiform encephalopathy (BSE) contaminated bovine tissues is considered as the origin of variant Creutzfeldt Jakob (vCJD) disease in human. To date, BSE agent is the only recognized zoonotic prion. Despite the variety of Transmissible Spongiform Encephalopathy (TSE) agents that have been circulating for centuries in farmed ruminants there is no apparent epidemiological link between exposure to ruminant products and the occurrence of other form of TSE in human like sporadic Creutzfeldt Jakob Disease (sCJD). However, the zoonotic potential of the diversity of circulating TSE agents has never been systematically assessed. The major issue in experimental assessment of TSEs zoonotic potential lies in the modeling of the ‘species barrier‘, the biological phenomenon that limits TSE agents’ propagation from a species to another. In the last decade, mice genetically engineered to express normal forms of the human prion protein has proved essential in studying human prions pathogenesis and modeling the capacity of TSEs to cross the human species barrier. To assess the zoonotic potential of prions circulating in farmed ruminants, we study their transmission ability in transgenic mice expressing human PrPC (HuPrP-Tg). Two lines of mice expressing different forms of the human PrPC (129Met or 129Val) are used to determine the role of the Met129Val dimorphism in susceptibility/resistance to the different agents. These transmission experiments confirm the ability of BSE prions to propagate in 129M- HuPrP-Tg mice and demonstrate that Met129 homozygotes may be susceptible to BSE in sheep or goat to a greater degree than the BSE agent in cattle and that these agents can convey molecular properties and neuropathological indistinguishable from vCJD. However homozygous 129V mice are resistant to all tested BSE derived prions independently of the originating species suggesting a higher transmission barrier for 129V-PrP variant. Transmission data also revealed that several scrapie prions propagate in HuPrP-Tg mice with efficiency comparable to that of cattle BSE. While the efficiency of transmission at primary passage was low, subsequent passages resulted in a highly virulent prion disease in both Met129 and Val129 mice. Transmission of the different scrapie isolates in these mice leads to the emergence of prion strain phenotypes that showed similar characteristics to those displayed by MM1 or VV2 sCJD prion. These results demonstrate that scrapie prions have a zoonotic potential and raise new questions about the possible link between animal and human prions. http://www.tandfonline.com/na101/home/literatum/publisher/tandf/journals/ content/kprn20/2016/kprn20.v010.sup01/19336896.2016.1163048/2016041 8/19336896.2016.1163048.fp.png_v03


http://www.tandfonline.com/doi/abs/10.1080/19336896.2016.1163048? journalCode=kprn20 Research Project: TRANSMISSION, DIFFERENTIATION, AND PATHOBIOLOGY OF TRANSMISSIBLE SPONGIFORM ENCEPHALOPATHIES Title: Transmission of scrapie prions to primate after an extended silent incubation period Authors item Comoy, Emmanuel - item Mikol, Jacqueline - item Luccantoni-Freire, Sophie - item Correia, Evelyne - item Lescoutra-Etchegaray, Nathalie item Durand, Valérie - item Dehen, Capucine - item Andreoletti, Olivier item Casalone, Cristina - item Richt, Juergen item Greenlee, Justin item Baron, Thierry - item Benestad, Sylvie - item Hills, Bob - item Brown, Paul - item Deslys, Jean-Philippe Submitted to: Scientific Reports Publication Type: Peer Reviewed Journal Publication Acceptance Date: May 28, 2015 Publication Date: June 30, 2015 Citation: Comoy, E.E., Mikol, J., Luccantoni-Freire, S., Correia, E., Lescoutra-Etchegaray, N., Durand, V., Dehen, C., Andreoletti, O., Casalone, C., Richt, J.A., Greenlee, J.J., Baron, T., Benestad, S., Brown, P., Deslys, J. 2015. Transmission of scrapie prions to primate after an extended silent incubation period. Scientific Reports. 5:11573. Interpretive Summary: The transmissible spongiform encephalopathies (also called prion diseases) are fatal neurodegenerative diseases that affect animals and humans. The agent of prion diseases is a misfolded form of the prion protein that is resistant to breakdown by the host cells. Since all mammals express prion protein on the surface of various cells such as neurons, all mammals are, in theory, capable of replicating prion diseases. One example of a prion disease, bovine spongiform encephalopathy (BSE; also called mad cow disease), has been shown to infect cattle, sheep, exotic undulates, cats, non-human primates, and humans when the new host is exposed to feeds or foods contaminated with the disease agent. The purpose of this study was to test whether nonhuman primates (cynomologous macaque) are susceptible to the agent of sheep scrapie. After an incubation period of approximately 10 years a macaque developed progressive clinical signs suggestive of neurologic disease. Upon postmortem examination and microscopic examination of tissues, there was a widespread distribution of lesions consistent with a transmissible spongiform encephalopathy. This information will have a scientific impact since it is the first study that demonstrates the transmission of scrapie to a non-human primate with a close genetic relationship to humans. This information is especially useful to regulatory officials and those involved with risk assessment of the potential transmission of animal prion diseases to humans. Technical Abstract: Classical bovine spongiform encephalopathy (c-BSE) is an animal prion disease that also causes variant Creutzfeldt-Jakob disease in humans. Over the past decades, c-BSE's zoonotic potential has been the driving force in establishing extensive protective measures for animal and human health. *** In complement to the recent demonstration that humanized mice are susceptible to scrapie, we report here the first observation of direct transmission of a natural classical scrapie isolate to a macaque after a 10year incubation period. Neuropathologic examination revealed all of the features of a prion disease: spongiform change, neuronal loss, and accumulation of PrPres throughout the CNS. *** This observation strengthens the questioning of the harmlessness of scrapie to humans, at a time when protective measures for human and animal health are being dismantled and reduced as c-BSE is considered controlled and being eradicated. *** Our results underscore the importance of precautionary and protective measures and the necessity for long-term experimental transmission studies to assess the zoonotic potential of other animal prion strains. http://www.ars.usda.gov/research/publications/publications.htm? SEQ_NO_115=313160 2015 O.05: Transmission of prions to primates after extended silent incubation periods: Implications for BSE and scrapie risk assessment in human populations


Emmanuel Comoy, Jacqueline Mikol, Valerie Durand, Sophie Luccantoni, Evelyne Correia, Nathalie Lescoutra, Capucine Dehen, and Jean-Philippe Deslys Atomic Energy Commission; Fontenay-aux-Roses, France Prion diseases (PD) are the unique neurodegenerative proteinopathies reputed to be transmissible under field conditions since decades. The transmission of Bovine Spongiform Encephalopathy (BSE) to humans evidenced that an animal PD might be zoonotic under appropriate conditions. Contrarily, in the absence of obvious (epidemiological or experimental) elements supporting a transmission or genetic predispositions, PD, like the other proteinopathies, are reputed to occur spontaneously (atpical animal prion strains, sporadic CJD summing 80% of human prion cases). Non-human primate models provided the first evidences supporting the transmissibiity of human prion strains and the zoonotic potential of BSE. Among them, cynomolgus macaques brought major information for BSE risk assessment for human health (Chen, 2014), according to their phylogenetic proximity to humans and extended lifetime. We used this model to assess the zoonotic potential of other animal PD from bovine, ovine and cervid origins even after very long silent incubation periods. *** We recently observed the direct transmission of a natural classical scrapie isolate to macaque after a 10-year silent incubation period, ***with features similar to some reported for human cases of sporadic CJD, albeit requiring fourfold long incubation than BSE. Scrapie, as recently evoked in humanized mice (Cassard, 2014), ***is the third potentially zoonotic PD (with BSE and L-type BSE), ***thus questioning the origin of human sporadic cases. We will present an updated panorama of our different transmission studies and discuss the implications of such extended incubation periods on risk assessment of animal PD for human health. =============== ***thus questioning the origin of human sporadic cases*** =============== ***our findings suggest that possible transmission risk of H-type BSE to sheep and human. Bioassay will be required to determine whether the PMCA products are infectious to these animals. ============== https://prion2015.files.wordpress.com/2015/05/prion2015abstracts.pdf Tuesday, December 16, 2014 *** Evidence for zoonotic potential of ovine scrapie prions Hervé Cassard,1, n1 Juan-Maria Torres,2, n1 Caroline Lacroux,1, JeanYves Douet,1, Sylvie L. Benestad,3, Frédéric Lantier,4, Séverine Lugan,1, Isabelle Lantier,4, Pierrette Costes,1, Naima Aron,1, Fabienne Reine,5, Laetitia Herzog,5, Juan-Carlos Espinosa,2, Vincent Beringue5, & Olivier Andréoletti1, Affiliations Contributions Corresponding author Journal name: Nature Communications Volume: 5, Article number: 5821 DOI: doi:10.1038/ncomms6821 Received 07 August 2014 Accepted 10 November 2014 Published 16 December 2014 Article tools Citation Reprints Rights & permissions Article metrics Abstract Although Bovine Spongiform Encephalopathy (BSE) is the cause of variant Creutzfeldt Jakob disease (vCJD) in humans, the zoonotic potential of scrapie prions remains unknown. Mice genetically engineered to overexpress the human prion protein (tgHu) have emerged as highly relevant models for gauging the capacity of prions to transmit to humans. These models can propagate human prions without any apparent transmission barrier and have been used used to confirm the zoonotic ability of BSE. Here we show that a panel of sheep scrapie prions transmit to several tgHu mice models with an efficiency comparable to that of cattle BSE. ***The serial transmission of different scrapie isolates in these mice led to the propagation of prions that are phenotypically identical to those causing sporadic CJD (sCJD) in humans. ***These results demonstrate that scrapie prions have a zoonotic potential


and raise new questions about the possible link between animal and human prions. Subject terms: Biological sciences• Medical research At a glance http://www.nature.com/ncomms/2014/141216/ncomms6821/full/ncomms6 821.html see more here ; http://www.nature.com/ncomms/2014/141216/ncomms6821/extref/ncomm s6821-s1.pdf why do we not want to do TSE transmission studies on chimpanzees $ 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 http://collections.europarchive.org/tna/20080102222950/http://www.bseinq uiry.gov.uk/files/yb/1990/09/23001001.pdf Wednesday, February 16, 2011 IN CONFIDENCE SCRAPIE TRANSMISSION TO CHIMPANZEES IN CONFIDENCE http://scrapie-usa.blogspot.com/2011/02/in-confidence-scrapietransmission-to.html Sunday, December 12, 2010 EFSA reviews BSE/TSE infectivity in small ruminant tissues News Story 2 December 2010 http://transmissiblespongiformencephalopathy.blogspot.com/2010/12/efsareviews-bsetse-infectivity-in.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 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 http://transmissiblespongiformencephalopathy.blogspot.com/2010/12/mole cular-typing-of-protease-resistant.html Thursday, November 18, 2010 Increased susceptibility of human-PrP transgenic mice to bovine spongiform encephalopathy following passage in sheep http://bse-atypical.blogspot.com/2010/11/increased-susceptibility-ofhuman-prp.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


Friday, February 11, 2011 Atypical/Nor98 Scrapie Infectivity in Sheep Peripheral Tissues http://nor-98.blogspot.com/2011/02/atypicalnor98-scrapie-infectivity-in.html 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-spreadfrom.html Wednesday, April 4, 2012 20120402 - Breach of quarantine/Violation de la mise en quarantaine of an ongoing Scrapie investigation http://transmissiblespongiformencephalopathy.blogspot.com/2012/04/2012 0402-breach-of-quarantineviolation.html Michigan and California have had a high spike in Goat Scrapie cases, compared to elsewhere ??? Tuesday, February 01, 2011 Sparse PrP-Sc accumulation in the placentas of goats with naturally acquired scrapie (Figure 6) including five goat cases in FY 2008 that originated from the same herd in Michigan. This is highly unusual for goats, and I strenuously urge that there should be an independent investigation into finding the common denominator for these 5 goats in the same herd in Michigan with Scrapie. ... http://scrapie-usa.blogspot.com/2011/02/sparse-prp-sc-accumulation-inplacentas.html Thursday, February 23, 2012 Atypical Scrapie NOR-98 confirmed Alberta Canada sheep January 2012 http://transmissiblespongiformencephalopathy.blogspot.com/2012/02/atypi cal-scrapie-nor-98-confirmed.html RESEARCH Emerging Infectious Diseases • www.cdc.gov/eid • Vol. 17, No. 5, May 2011 Experimental Oral Transmission of Atypical Scrapie to Sheep Marion M. Simmons, S. Jo Moore,1 Timm Konold, Lisa Thurston, Linda A. Terry, Leigh Thorne, Richard Lockey, Chris Vickery, Stephen A.C. Hawkins, Melanie J. Chaplin, and John Spiropoulos To investigate the possibility of oral transmission of atypical scrapie in sheep and determine the distribution of infectivity in the animals’ peripheral tissues, we challenged neonatal lambs orally with atypical scrapie; they were then killed at 12 or 24 months. Screening test results were negative for disease-specifi c prion protein in all but 2 recipients; they had positive results for examination of brain, but negative for peripheral tissues. Infectivity of brain, distal ileum, and spleen from all animals was assessed in mouse bioassays; positive results were obtained from tissues that had negative results on screening. These fi ndings demonstrate that atypical scrapie can be transmitted orally and indicate that it has the potential for natural transmission and iatrogenic spread through animal feed. Detection of infectivity in tissues negative by current surveillance methods indicates that diagnostic sensitivity is suboptimal for atypical scrapie, and potentially infectious material may be able to pass into the human food chain. SNIP... Although we do not have epidemiologic evidence that supports the effi cient spread of disease in the fi eld, these data imply that disease is potentially transmissible under fi eld situations and that spread through animal feed may be possible if the current feed restrictions were to be relaxed. Additionally, almost no data are available on the potential for


atypical scrapie to transmit to other food animal species, certainly by the oral route. However, work with transgenic mice has demonstrated the potential susceptibility of pigs, with the disturbing fi nding that the biochemical properties of the resulting PrPSc have changed on transmission (40). The implications of this observation for subsequent transmission and host target range are currently unknown. How reassuring is this absence of detectable PrPSc from a public health perspective? The bioassays performed in this study are not titrations, so the infectious load of the positive gut tissues cannot be quantifi ed, although infectivity has been shown unequivocally. No experimental data are currently available on the zoonotic potential of atypical scrapie, either through experimental challenge of humanized mice or any meaningful epidemiologic correlation with human forms of TSE. However, the detection of infectivity in the distal ileum of animals as young as 12 months, in which all the tissues tested were negative for PrPSc by the currently available screening and confi rmatory diagnostic tests, indicates that the diagnostic sensitivity of current surveillance methods is suboptimal for detecting atypical scrapie and that potentially infectious material may be able to pass into the human food chain undetected. Emerging Infectious Diseases • www.cdc.gov/eid • Vol. 17, No. 5, May 2011 http://wwwnc.cdc.gov/eid/article/17/5/pdfs/10-1654.pdf 1: J Infect Dis 1980 Aug;142(2):205-8 Oral transmission of kuru, Creutzfeldt-Jakob disease, and scrapie to nonhuman primates. Gibbs CJ Jr, Amyx HL, Bacote A, Masters CL, Gajdusek DC. Kuru and Creutzfeldt-Jakob disease of humans and scrapie disease of sheep and goats were transmitted to squirrel monkeys (Saimiri sciureus) that were exposed to the infectious agents only by their nonforced consumption of known infectious tissues. The asymptomatic incubation period in the one monkey exposed to the virus of kuru was 36 months; that in the two monkeys exposed to the virus of Creutzfeldt-Jakob disease was 23 and 27 months, respectively; and that in the two monkeys exposed to the virus of scrapie was 25 and 32 months, respectively. Careful physical examination of the buccal cavities of all of the monkeys failed to reveal signs or oral lesions. One additional monkey similarly exposed to kuru has remained asymptomatic during the 39 months that it has been under observation. snip... The successful transmission of kuru, Creutzfeldt-Jakob disease, and scrapie by natural feeding to squirrel monkeys that we have reported provides further grounds for concern that scrapie-infected meat may occasionally give rise in humans to Creutzfeldt-Jakob disease. PMID: 6997404 http://www.ncbi.nlm.nih.gov/entrez/query.fcgi? cmd=Retrieve&db=PubMed&list_uids=6997404&dopt=Abstract Recently the question has again been brought up as to whether scrapie is transmissible to man. This has followed reports that the disease has been transmitted to primates. One particularly lurid speculation (Gajdusek 1977) conjectures that the agents of scrapie, kuru, Creutzfeldt-Jakob disease and transmissible encephalopathy of mink are varieties of a single "virus". The U.S. Department of Agriculture concluded that it could "no longer justify or permit scrapie-blood line and scrapie-exposed sheep and goats to be processed for human or animal food at slaughter or rendering plants" (ARC 84/77)" The problem is emphasised by the finding that some strains of scrapie produce lesions identical to the once which characterise the human dementias" Whether true or not. the hypothesis that these agents might be transmissible to man raises two considerations. First, the safety of laboratory personnel requires prompt attention. Second, action such as the "scorched meat" policy of USDA makes the solution of the acrapie problem urgent if the sheep industry is not to suffer grievously. snip... 76/10.12/4.6 http://web.archive.org/web/20010305223125/www.bseinquiry.gov.uk/files/y


b/1976/10/12004001.pdf Nature. 1972 Mar 10;236(5341):73-4. Transmission of scrapie to the cynomolgus monkey (Macaca fascicularis). Gibbs CJ Jr, Gajdusek DC. Nature 236, 73 - 74 (10 March 1972); doi:10.1038/236073a0 Transmission of Scrapie to the Cynomolgus Monkey (Macaca fascicularis) C. J. GIBBS jun. & D. C. GAJDUSEK National Institute of Neurological Diseases and Stroke, National Institutes of Health, Bethesda, Maryland SCRAPIE has been transmitted to the cynomolgus, or crab-eating, monkey (Macaca fascicularis) with an incubation period of more than 5 yr from the time of intracerebral inoculation of scrapie-infected mouse brain. The animal developed a chronic central nervous system degeneration, with ataxia, tremor and myoclonus with associated severe scrapie-like pathology of intensive astroglial hypertrophy and proliferation, neuronal vacuolation and status spongiosus of grey matter. The strain of scrapie virus used was the eighth passage in Swiss mice (NIH) of a Compton strain of scrapie obtained as ninth intracerebral passage of the agent in goat brain, from Dr R. L. Chandler (ARC, Compton, Berkshire). http://www.nature.com/nature/journal/v236/n5341/abs/236073a0.html http://scrapie-usa.blogspot.com/2010/04/scrapie-and-atypical-scrapie.html BSE, SCRAPIE, CWD, TSE, PRION, and the legal trading therefrom, thanks to the USDA and OIE et al Tuesday, May 31, 2016 Priority Interim Position Paper PROTECTING THE FOOD CHAIN FROM PRIONS Perspectives http://transmissiblespongiformencephalopathy.blogspot.com/2016/05/priori ty-interim-position-paper.html Tuesday, June 07, 2016 Comparison of two US sheep scrapie isolates supports identification as separate strains Research Project: TRANSMISSION, DIFFERENTIATION, AND PATHOBIOLOGY OF TRANSMISSIBLE SPONGIFORM ENCEPHALOPATHIES http://scrapie-usa.blogspot.com/2016/06/comparison-of-two-us-sheepscrapie.html CHRONIC WASTING DISEASE TSE PRION PRION 2016 TOKYO Zoonotic Potential of CWD Prions: An Update Ignazio Cali1, Liuting Qing1, Jue Yuan1, Shenghai Huang2, Diane Kofskey1,3, Nicholas Maurer1, Debbie McKenzie4, Jiri Safar1,3,5, Wenquan Zou1,3,5,6, Pierluigi Gambetti1, Qingzhong Kong1,5,6 1Department of Pathology, 3National Prion Disease Pathology Surveillance Center, 5Department of Neurology, 6National Center for Regenerative Medicine, Case Western Reserve University, Cleveland, OH 44106, USA. 4Department of Biological Sciences and Center for Prions and Protein Folding Diseases, University of Alberta, Edmonton, Alberta, Canada, 2Encore Health Resources, 1331 Lamar St, Houston, TX 77010 Chronic wasting disease (CWD) is a widespread and highly transmissible prion disease in free-ranging and captive cervid species in North America. The zoonotic potential of CWD prions is a serious public health concern, but the susceptibility of human CNS and peripheral organs to CWD prions remains largely unresolved. We reported earlier that peripheral and CNS infections were detected in transgenic mice expressing human PrP129M


or PrP129V. Here we will present an update on this project, including evidence for strain dependence and influence of cervid PrP polymorphisms on CWD zoonosis as well as the characteristics of experimental human CWD prions. PRION 2016 TOKYO In Conjunction with Asia Pacific Prion Symposium 2016 PRION 2016 Tokyo Prion 2016 http://prion2016.org/dl/newsletter_03.pdf Prion 2016 Purchase options Price * Issue Purchase USD 198.00 http://www.tandfonline.com/toc/kprn20/10/sup1 Cervid to human prion transmission Kong, Qingzhong Case Western Reserve University, Cleveland, OH, United States Abstract Prion disease is transmissible and invariably fatal. Chronic wasting disease (CWD) is the prion disease affecting deer, elk and moose, and it is a widespread and expanding epidemic affecting 22 US States and 2 Canadian provinces so far. CWD poses the most serious zoonotic prion transmission risks in North America because of huge venison consumption (>6 million deer/elk hunted and consumed annually in the USA alone), significant prion infectivity in muscles and other tissues/fluids from CWD-affected cervids, and usually high levels of individual exposure to CWD resulting from consumption of the affected animal among often just family and friends. However, we still do not know whether CWD prions can infect humans in the brain or peripheral tissues or whether clinical/asymptomatic CWD zoonosis has already occurred, and we have no essays to reliably detect CWD infection in humans. We hypothesize that: (1) The classic CWD prion strain can infect humans at low levels in the brain and peripheral lymphoid tissues; (2) The cervid-to-human transmission barrier is dependent on the cervid prion strain and influenced by the host (human) prion protein (PrP) primary sequence; (3) Reliable essays can be established to detect CWD infection in humans;and (4) CWD transmission to humans has already occurred. We will test these hypotheses in 4 Aims using transgenic (Tg) mouse models and complementary in vitro approaches. Aim 1 will prove that the classical CWD strain may infect humans in brain or peripheral lymphoid tissues at low levels by conducting systemic bioassays in a set of "humanized" Tg mouse lines expressing common human PrP variants using a number of CWD isolates at varying doses and routes. Experimental "human CWD" samples will also be generated for Aim 3. Aim 2 will test the hypothesis that the cervid-to-human prion transmission barrier is dependent on prion strain and influenced by the host (human) PrP sequence by examining and comparing the transmission efficiency and phenotypes of several atypical/unusual CWD isolates/strains as well as a few prion strains from other species that have adapted to cervid PrP sequence, utilizing the same panel of humanized Tg mouse lines as in Aim 1. Aim 3 will establish reliable essays for detection and surveillance of CWD infection in humans by examining in details the clinical, pathological, biochemical and in vitro seeding properties of existing and future experimental "human CWD" samples generated from Aims 1-2 and compare them with those of common sporadic human Creutzfeldt-Jakob disease (sCJD) prions. Aim 4 will attempt to detect clinical CWD-affected human cases by


examining a significant number of brain samples from prion-affected human subjects in the USA and Canada who have consumed venison from CWD-endemic areas utilizing the criteria and essays established in Aim 3. The findings from this proposal will greatly advance our understandings on the potential and characteristics of cervid prion transmission in humans, establish reliable essays for CWD zoonosis and potentially discover the first case(s) of CWD infection in humans. Public Health Relevance There are significant and increasing human exposure to cervid prions because chronic wasting disease (CWD, a widespread and highly infectious prion disease among deer and elk in North America) continues spreading and consumption of venison remains popular, but our understanding on cervid-to-human prion transmission is still very limited, raising public health concerns. This proposal aims to define the zoonotic risks of cervid prions and set up and apply essays to detect CWD zoonosis using mouse models and in vitro methods. The findings will greatly expand our knowledge on the potentials and characteristics of cervid prion transmission in humans, establish reliable essays for such infections and may discover the first case(s) of CWD infection in humans. Funding Agency Agency National Institute of Health (NIH) Institute National Institute of Neurological Disorders and Stroke (NINDS) Type Research Project (R01) Project # 1R01NS088604-01A1 Application # 9037884 Study Section Cellular and Molecular Biology of Neurodegeneration Study Section (CMND) Program Officer Wong, May Project Start 2015-09-30 Project End 2019-07-31 Budget Start 2015-09-30 Budget End 2016-07-31 Support Year 1 Fiscal Year 2015 Total Cost $337,507 Indirect Cost $118,756 Institution Name Case Western Reserve University Department Pathology Type Schools of Medicine DUNS # 077758407 City Cleveland State OH Country United States Zip Code 44106 http://grantome.com/grant/NIH/R01-NS088604-01A1 ===================================================== ====== We hypothesize that: (1) The classic CWD prion strain can infect humans at low levels in the brain and peripheral lymphoid tissues; (2) The cervid-to-human transmission barrier is dependent on the cervid


prion strain and influenced by the host (human) prion protein (PrP) primary sequence; (3) Reliable essays can be established to detect CWD infection in humans;and (4) *** CWD transmission to humans has already occurred. *** We will test these hypotheses in 4 Aims using transgenic (Tg) mouse models and complementary in vitro approaches. ===================================================== ======= Key Molecular Mechanisms of TSEs Zabel, Mark D. Colorado State University-Fort Collins, Fort Collins, CO, United States Abstract Prion diseases, or transmissible spongiform encephalopathies (TSEs), are fatal neurodegenerative diseases affecting humans, cervids, bovids, and ovids. The absolute requirement of PrPC expression to generate prion diseases and the lack of instructional nucleic acid define prions as unique infectious agents. Prions exhibit species-specific tropism, inferring that unique prion strains exist that preferentially infct certain host species and confront transmission barriers to heterologous host species. However, transmission barriers are not absolute. Scientific consensus agrees that the sheep TSE scrapie probably breached the transmission barrier to cattle causing bovine spongiform encephalopathy that subsequently breached the human transmission barrier and likely caused several hundred deaths by a new-variant form of the human TSE Creutzfeldt-Jakob disease in the UK and Europe. The impact to human health, emotion and economies can still be felt in areas like farming, blood and organ donations and the threat of a latent TSE epidemic. This precedent raises the real possibility of other TSEs, like chronic wasting disease of cervids, overcoming similar human transmission barriers. A groundbreaking discovery made last year revealed that mice infected with heterologous prion strains facing significant transmission barriers replicated prions far more readily in spleens than brains6. Furthermore, these splenic prions exhibited weakened transmission barriers and expanded host ranges compared to neurogenic prions. These data question conventional wisdom of avoiding neural tissue to avoid prion xenotransmission, when more promiscuous prions may lurk in extraneural tissues. Data derived from work previously funded by NIH demonstrate that Complement receptors CD21/35 bind prions and high density PrPC and differentially impact prion disease depending on the prion isolate or strain used. Recent advances in live animal and whole organ imaging have led us to generate preliminary data to support novel, innovative approaches to assessing prion capture and transport. We plan to test our unifying hypothesis for this proposal that CD21/35 control the processes of peripheral prion capture, transport, strain selection and xenotransmission in the following specific aims. 1. Assess the role of CD21/35 in splenic prion strain selection and host range expansion. 2. Determine whether CD21/35 and C1q differentially bind distinct prion strains 3. Monitor the effects of CD21/35 on prion trafficking in real time and space 4. Assess the role of CD21/35 in incunabular prion trafficking Public Health Relevance Transmissible spongiform encephalopathies, or prion diseases, are devastating illnesses that greatly impact public health, agriculture and wildlife in North America and around the world. The impact to human health, emotion and economies can still be felt in areas like farming, blood and organ donations and the threat of a latent TSE epidemic. This precedent raises the real possibility of other TSEs, like chronic wasting disease (CWD) of cervids, overcoming similar human transmission barriers. Early this year Canada reported its first case of BSE in over a decade audits first case of CWD in farmed elk in three years, underscoring the need for continued vigilance and research. Identifying mechanisms of transmission and zoonoses remains an extremely important and intense area of research that will benefit human and other animal populations. Funding Agency Agency National Institute of Health (NIH) Institute National Institute of Allergy and Infectious Diseases (NIAID) Type High Priority, Short Term Project Award (R56) Project # 1R56AI122273-01A1 Application # 9211114 Study Section Cellular and Molecular Biology of Neurodegeneration Study


Section (CMND) Program Officer Beisel, Christopher E Project Start 2016-02-16 Project End 2017-01-31 Budget Start 2016-02-16 Budget End 2017-01-31 Support Year 1 Fiscal Year 2016 Total Cost Indirect Cost Institution Name Colorado State University-Fort Collins Department Microbiology/Immun/Virology Type Schools of Veterinary Medicine DUNS # 785979618 City Fort Collins State CO Country United States Zip Code 80523 http://grantome.com/grant/NIH/R56-AI122273-01A1 PMCA Detection of CWD Infection in Cervid and Non-Cervid Species Hoover, Edward Arthur Colorado State University-Fort Collins, Fort Collins, CO, United States Abstract Chronic wasting disease (CWD) of deer and elk is an emerging highly transmissible prion disease now recognized in 18 States, 2 Canadian provinces, and Korea. We have shown that Infected deer harbor and shed high levels of infectious prions in saliva, blood, urine, and feces, and in the tissues generating those body fluids and excreta, thereby leading to facile transmission by direct contact and environmental contamination. We have also shown that CWD can infect some non-cervid species, thus the potential risk CWD represents to domestic animal species and to humans remains unknown. Whether prions borne in blood, saliva, nasal fluids, milk, or excreta are generated or modified in the proximate peripheral tissue sites, may differ in subtle ways from those generated in brain, or may be adapted for mucosal infection remain open questions. The increasing parallels in the pathogenesis between prion diseases and human neurodegenerative conditions, such as Alzheimer's and Parkinson's diseases, add relevance to CWD as a transmissible protein misfolding disease. The overall goal of this work is to elucidate the process of CWD prion transmission from mucosal secretory and excretory tissue sites by addressing these questions: (a) What are the kinetics and magnitude of CWD prion shedding post-exposure? (b) Are excreted prions biochemically distinct, or not, from those in the CNS? (c) Are peripheral epithelial or CNS tissues, or both, the source of excreted prions? and (d) Are excreted prions adapted for horizontal transmission via natural/transmucosal routes? The specific aims of this proposal are: (1) To determine the onset and consistency of CWD prion shedding in deer and cervidized mice; (2); To compare the biochemical and biophysical properties of excretory vs. CNS prions; (3) To determine the capacity of peripheral tissues to support replication of CWD prions; (4) To determine the protease- sensitive infectious fraction of excreted vs. CNS prions; and (5) To compare the mucosal infectivity of excretory vs. CNS prions. Understanding the mechanisms that enable efficient prion dissemination and shedding will help elucidate how horizontally transmissible prions evolve and succeed, and is the basis of this proposal. Understanding how infectious misfolded proteins (prions) are generated, trafficked, shed, and transmitted will aid in preventing, treating, and managing the risks associated with these agents and the diseases they cause. Public Health Relevance Chronic wasting disease (CWD) of deer and elk is an emergent highly transmissible prion disease now recognized throughout the USA as well as in Canada and Korea. We have shown that infected deer harbor and shed high levels of infectious prions in saliva, blood, urine, and feces thereby leading to transmission by direct contact and environmental contamination. In that our studies have also shown that


CWD can infect some non-cervid species, the potential risk CWD may represents to domestic animal species and humans remains unknown. The increasing parallels in the development of major human neurodegenerative conditions, such as Alzheimer's and Parkinson's diseases, and prion diseases add relevance to CWD as a model of a transmissible protein misfolding disease. Understanding how infectious misfolded proteins (prions) are generated and transmitted will aid in interrupting, treating, and managing the risks associated with these agents and the diseases they cause. Funding Agency Agency National Institute of Health (NIH) Institute National Institute of Neurological Disorders and Stroke (NINDS) Type Research Project (R01) Project # 4R01NS061902-07 Application # 9010980 Study Section Cellular and Molecular Biology of Neurodegeneration Study Section (CMND) Program Officer Wong, May Project Start 2009-09-30 Project End 2018-02-28 Budget Start 2016-03-01 Budget End 2017-02-28 Support Year 7 Fiscal Year 2016 Total Cost $409,868 Indirect Cost $134,234 Institution Name Colorado State University-Fort Collins Department Microbiology/Immun/Virology Type Schools of Veterinary Medicine DUNS # 785979618 City Fort Collins State CO Country United States Zip Code 80523 http://grantome.com/grant/NIH/R01-NS061902-07 LOOKING FOR CWD IN HUMANS AS nvCJD or as an ATYPICAL CJD, LOOKING IN ALL THE WRONG PLACES $$$ *** These results would seem to suggest that CWD does indeed have zoonotic potential, at least as judged by the compatibility of CWD prions and their human PrPC target. Furthermore, extrapolation from this simple in vitro assay suggests that if zoonotic CWD occurred, it would most likely effect those of the PRNP codon 129-MM genotype and that the PrPres type would be similar to that found in the most common subtype of sCJD (MM1).*** https://www.landesbioscience.com/journals/prion/article/28124/? nocache=112223249 PRION 2015 CONFERENCE FT. COLLINS CWD RISK FACTORS TO HUMANS *** LATE-BREAKING ABSTRACTS PRION 2015 CONFERENCE *** O18 Zoonotic Potential of CWD Prions Liuting Qing1, Ignazio Cali1,2, Jue Yuan1, Shenghai Huang3, Diane Kofskey1, Pierluigi Gambetti1, Wenquan Zou1, Qingzhong Kong1 1Case Western Reserve University, Cleveland, Ohio, USA, 2Second University of Naples, Naples, Italy, 3Encore Health Resources, Houston, Texas, USA


*** These results indicate that the CWD prion has the potential to infect human CNS and peripheral lymphoid tissues and that there might be asymptomatic human carriers of CWD infection. ================== ***These results indicate that the CWD prion has the potential to infect human CNS and peripheral lymphoid tissues and that there might be asymptomatic human carriers of CWD infection.*** ================== P.105: RT-QuIC models trans-species prion transmission Kristen Davenport, Davin Henderson, Candace Mathiason, and Edward Hoover Prion Research Center; Colorado State University; Fort Collins, CO USA Conversely, FSE maintained sufficient BSE characteristics to more efficiently convert bovine rPrP than feline rPrP. Additionally, human rPrP was competent for conversion by CWD and fCWD. ***This insinuates that, at the level of protein:protein interactions, the barrier preventing transmission of CWD to humans is less robust than previously estimated. ================ ***This insinuates that, at the level of protein:protein interactions, the barrier preventing transmission of CWD to humans is less robust than previously estimated.*** ================ https://prion2015.files.wordpress.com/2015/05/programguide1.pdf *** PRICE OF CWD TSE PRION POKER GOES UP 2014 *** Transmissible Spongiform Encephalopathy TSE PRION update January 2, 2014 *** chronic wasting disease, there was no absolute barrier to conversion of the human prion protein. *** Furthermore, the form of human PrPres produced in this in vitro assay when seeded with CWD, resembles that found in the most common human prion disease, namely sCJD of the MM1 subtype. http://wwwnc.cdc.gov/eid/article/20/1/13-0858_article.htm http://chronic-wasting-disease.blogspot.com/2014/01/molecular-barriersto-zoonotic.html *** These results would seem to suggest that CWD does indeed have zoonotic potential, at least as judged by the compatibility of CWD prions and their human PrPC target. Furthermore, extrapolation from this simple in vitro assay suggests that if zoonotic CWD occurred, it would most likely effect those of the PRNP codon 129-MM genotype and that the PrPres type would be similar to that found in the most common subtype of sCJD (MM1).*** https://www.landesbioscience.com/journals/prion/article/28124/? nocache=112223249 *** The potential impact of prion diseases on human health was greatly magnified by the recognition that interspecies transfer of BSE to humans by beef ingestion resulted in vCJD. While changes in animal feed constituents and slaughter practices appear to have curtailed vCJD, there is concern that CWD of free-ranging deer and elk in the U.S. might also cross the species barrier. Thus, consuming venison could be a source of human prion disease. Whether BSE and CWD represent interspecies scrapie transfer or are newly arisen prion diseases is unknown. Therefore, the possibility of transmission of prion disease through other food animals cannot be ruled out. There is evidence that vCJD can be transmitted through blood transfusion. There is likely a pool of unknown size of asymptomatic individuals infected with vCJD, and there may be asymptomatic individuals infected with the CWD equivalent. These circumstances represent a potential threat to blood, blood products, and plasma supplies.


http://cdmrp.army.mil/prevfunded/nprp/NPRP_Summit_Final_Report.pdf ***********CJD REPORT 1994 increased risk for consumption of veal and venison and lamb*********** CREUTZFELDT JAKOB DISEASE SURVEILLANCE IN THE UNITED KINGDOM THIRD ANNUAL REPORT AUGUST 1994 Consumption of venison and veal was much less widespread among both cases and controls. For both of these meats there was evidence of a trend with increasing frequency of consumption being associated with increasing risk of CJD. (not nvCJD, but sporadic CJD...tss) These associations were largely unchanged when attention was restricted to pairs with data obtained from relatives. ... Table 9 presents the results of an analysis of these data. There is STRONG evidence of an association between ‘’regular’’ veal eating and risk of CJD (p = .0.01). Individuals reported to eat veal on average at least once a year appear to be at 13 TIMES THE RISK of individuals who have never eaten veal. There is, however, a very wide confidence interval around this estimate. There is no strong evidence that eating veal less than once per year is associated with increased risk of CJD (p = 0.51). The association between venison eating and risk of CJD shows similar pattern, with regular venison eating associated with a 9 FOLD INCREASE IN RISK OF CJD (p = 0.04). There is some evidence that risk of CJD INCREASES WITH INCREASING FREQUENCY OF LAMB EATING (p = 0.02). The evidence for such an association between beef eating and CJD is weaker (p = 0.14). When only controls for whom a relative was interviewed are included, this evidence becomes a little STRONGER (p = 0.08). snip... It was found that when veal was included in the model with another exposure, the association between veal and CJD remained statistically significant (p = < 0.05 for all exposures), while the other exposures ceased to be statistically significant (p = > 0.05). snip... In conclusion, an analysis of dietary histories revealed statistical associations between various meats/animal products and INCREASED RISK OF CJD. When some account was taken of possible confounding, the association between VEAL EATING AND RISK OF CJD EMERGED AS THE STRONGEST OF THESE ASSOCIATIONS STATISTICALLY. ... snip... In the study in the USA, a range of foodstuffs were associated with an increased risk of CJD, including liver consumption which was associated with an apparent SIX-FOLD INCREASE IN THE RISK OF CJD. By comparing the data from 3 studies in relation to this particular dietary factor, the risk of liver consumption became non-significant with an odds ratio of 1.2 (PERSONAL COMMUNICATION, PROFESSOR A. HOFMAN. ERASMUS UNIVERSITY, ROTTERDAM). (???...TSS) snip...see full report ; http://collections.europarchive.org/tna/20090505194948/http://bseinquiry.g ov.uk/files/yb/1994/08/00004001.pdf 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/f iles/yb/1994/10/00003001.pdf Monday, May 02, 2016 *** Zoonotic Potential of CWD Prions: An Update Prion 2016 Tokyo *** http://chronic-wasting-disease.blogspot.com/2016/05/zoonotic-potential-ofcwd-prions-update.html *** PRION 2014 CONFERENCE CHRONIC WASTING DISEASE CWD http://chronic-wasting-disease.blogspot.com/2014/06/prion-2014-chronicwasting-disease-cwd.html *** PPo3-7: Prion Transmission from Cervids to Humans is Straindependent *** 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 straindependent and humans can be vulnerable to novel cervid prion strains. PPo2-27: Generation of a Novel form of Human PrPSc by Inter-species Transmission of Cervid Prions *** 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 *** The data presented here substantiate and expand previous reports on the existence of different CWD strains. https://www.landesbioscience.com/journals/prion/Prion4-3-PPo2.pdf Envt.07: Pathological Prion Protein (PrPTSE) in Skeletal Muscles of Farmed and Free Ranging White-Tailed Deer Infected with Chronic Wasting Disease ***The presence and seeding activity of PrPTSE in skeletal muscle from CWD-infected cervids suggests prevention of such tissue in the human diet as a precautionary measure for food safety, pending on further


clarification of whether CWD may be transmissible to humans. http://www.landesbioscience.com/journals/prion/Prion5-SuppPrionEnvironment.pdf?nocache=1333529975 >>>CHRONIC WASTING DISEASE , THERE WAS NO ABSOLUTE BARRIER TO CONVERSION OF THE HUMAN PRION PROTEIN>> It is distinct from atypical BSE, which may develop spontaneously, according to information from the U.S. Centers for Disease Control and Prevention. THIS IS A MYTH $$$ ***atypical spontaneous BSE in France LOL*** FRANCE STOPS TESTING FOR MAD COW DISEASE BSE, and here’s why, to many spontaneous events of mad cow disease $$$ ***so 20 cases of atypical BSE in France, compared to the remaining 40 cases in the remaining 12 Countries, divided by the remaining 12 Countries, about 3+ cases per country, besides Frances 20 cases. you cannot explain this away with any spontaneous BSe. ...TSS Sunday, October 5, 2014 France stops BSE testing for Mad Cow Disease http://transmissiblespongiformencephalopathy.blogspot.com/2014/10/franc e-stops-bse-testing-for-mad-cow.html Thursday, March 24, 2016 FRANCE CONFIRMS BOVINE SPONGIFORM ENCEPHALOPATHY BSE MAD COW (ESB) chez une vache dans les Ardennes http://bovineprp.blogspot.com/2016/03/france-confirms-bovinespongiform.html ***atypical spontaneous BSE in France LOL*** FRANCE STOPS TESTING FOR MAD COW DISEASE BSE, and here’s why, to many spontaneous events of mad cow disease $$$ If you Compare France to other Countries with atypical BSE, in my opinion, you cannot explain this with ‘spontaneous’. Table 1: Number of Atypical BSE cases reported by EU Member States in the period 2001–2014 by country and by type (L- and H-BSE) (extracted from EU BSE databases on 1 July 2014). By 2015, these data might be more comprehensive following a request from the European Commission to Member States for re-testing and retrospective classification of all positive bovine isolates in the EU in the years 2003–2009 BSE type Country 2001 2002 2003 2004 2005 2006 2007 2008 2009 2010 2011 2012 2013(a) 2014(a) Total H-BSE Austria 1 1 France(b) 1 2 3 1 2 2 2 2 15 Germany 1 1 2 Ireland 1 1 2 1 5 The Netherlands 1 1 Poland 1 1 2 Portugal 1 1 Spain 1 1 2 Sweden 1 1 United Kingdom 1 1 1 1 1 5 Total 2 3 3 1 1 2 2 2 4 4 5 1 4 1 35 L-BSE Austria 1 1 2 Denmark 1 1 France(b) 1 1 1 1 2 1 3 2 1 1 14 Germany 1 1 2


Italy 1 1 1 1 1 5 The Netherlands 1 1 1 3 Poland 1 2 2 1 2 1 2 1 12 Spain 2 2 United Kingdom 1 1 1 1 4 Total 0 5 3 4 3 3 6 3 3 4 3 6 1 1 45 Total Atypical cases (H + L) 2 8 6 5 4 5 8 5 7 8 8 7 5 2 80 (a): Data for 2013-2014 are incomplete and may not include all cases/countries reported. (b): France has performed extensive retrospective testing to classify BSE cases, which is probably the explanation for the higher number of Atypical BSE cases reported in this country. The number of Atypical BSE cases detected in countries that have already identified them seems to be similar from year to year. In France, a retrospective study of all TSE-positive cattle identified through the compulsory EU surveillance between 2001 and 2007 indicated that the prevalence of H-BSE and L-BSE was 0.35 and 0.41 cases per million adult cattle tested, respectively, which increased to 1.9 and 1.7 cases per million, respectively, in tested animals over eight years old (Biacabe et al., 2008). No comprehensive study on the prevalence of Atypical BSE cases has yet been carried out in other EU Member States. All cases of Atypical BSE reported in the EU BSE databases have been identified by active surveillance testing (59 % in fallen stock, 38 % in healthy slaughtered cattle and 4 % in emergency slaughtered cattle). Cases were reported in animals over eight years of age, with the exception of two cases (one HBSE and one L-BSE) detected in Spain in 2011/2012. One additional case of H-BSE was detected in Switzerland in 2012 in a cow born in Germany in 2005 (Guldimann et al., 2012). http://www.efsa.europa.eu/sites/default/files/scientific_output/files/main_do cuments/3798.pdf http://transmissiblespongiformencephalopathy.blogspot.com/2014/10/franc e-stops-bse-testing-for-mad-cow.html SPONTANEOUS TRANSMISSIBLE SPONGIFORM ENCEPHALOPATHY TSE PRION AKA MAD COW TYPE DISEASE ??? *** We describe the transmission of spongiform encephalopathy in a nonhuman primate inoculated 10 years earlier with a strain of sheep c-scrapie. Because of this extended incubation period in a facility in which other prion diseases are under study, we are obliged to consider two alternative possibilities that might explain its occurrence. We first considered the possibility of a sporadic origin (like CJD in humans). Such an event is extremely improbable because the inoculated animal was 14 years old when the clinical signs appeared, i.e. about 40% through the expected natural lifetime of this species, compared to a peak age incidence of 60– 65 years in human sporadic CJD, or about 80% through their expected lifetimes. ***Moreover, sporadic disease has never been observed in breeding colonies or primate research laboratories, most notably among hundreds of animals over several decades of study at the National Institutes of Health25, and in nearly twenty older animals continuously housed in our own facility.*** >>> Moreover, sporadic disease has never been observed in breeding colonies or primate research laboratories, most notably among hundreds of animals over several decades of study at the National Institutes of Health25, and in nearly twenty older animals continuously housed in our own facility.