Thursday, April 11, 2013

Scientists develop new test for BSE risk materials i.e. SRM's

Scientists develop new test for BSE risk materials



Florida State University | Updated: 04/10/2013



A new screening method to detect tissues associated with Bovine Spongiform Encephalopathy (BSE) in processed food will be developed through a new license agreement between Florida State University and Gainesville-based ELISA Technologies.



Invented by Peggy Hsieh, a food scientist and the Betty M. Watts professor in FSU’s Department of Nutrition, Food and Exercise Sciences, the screening method uses a novel monoclonal antibody to detect the presence of bovine central nervous system (CNS) tissue in processed food and animal feed products. Consumption of foods containing infected bovine CNS tissue is believed to be the main cause of the extremely rare transmission of the disease to humans. The U.S. Food and Drug Administration has banned this risk-related material in human food since 2004, and in animal feed since 2009.



“While we have been lucky enough to have avoided any recent outbreaks of this deadly disease, it remains a serious concern both here in the U.S. and especially overseas in Europe,” Hsieh said. “This new test kit aims to address some of those concerns by providing a low-cost, accurate screening method that would allow for the routine testing of heat-processed foods and feed products.”



The main advantage of Hsieh’s new testing kit is the ability of the antibody to detect the presence of CNS tissue even after the food has been exposed to high levels of heat. Currently, there are no useful testing methods for food that has been heat processed, creating potential situations where contaminated tissue could slip though established detection protocols. Hsieh’s invention also will be important in the testing of animal feed products in order to prevent the introduction of disease contaminated CNS tissue into animal populations.



Laura Allred, president of ELISA Technologies, reports that “the antibody developed by Dr. Hsieh is incredibly sensitive and robust, creating an assay that can detect as little as 2 parts per million (ppm) of the target protein. This method will be a valuable tool for industry and regulators.”



“Dr. Hsieh has developed an impressive reputation for her work in the areas of food safety and quality, and I commend her efforts to transform her work into market-viable products,” said Gary K. Ostrander, vice president for Research. “This type of commercialization is a great example of the economic value of university-based research and the immediate benefit it can bring to the public.”



To learn more about FSU’s research commercialization efforts, visit the Office of IP Development and Commercialization. To learn more about the company developing the new test kits, visit ELISA Technologies.









thank you !



better late than never I suppose, but the mad cows have been out of the barn for some time now $$$





SOME other interesting reads on the TSE PRION aka mad cow SRM debacle...





FLORIDA STATE UNIVERSITY COLLEGE OF HUMAN SCIENCES IMMUNODETECTION OF BOVINE CENTRAL NERVOUS SYSTEM TISSUE IN PROCESSED PRODUCTS By QINCHUN RAO A Dissertation submitted to the Department of Nutrition, Food and Exercise Sciences in partial fulfillment of the requirements for the degree of Doctoral of Philosophy Degree Awarded: Summer Semester, 2009
















Wednesday, March 27, 2013


U-turn considered on European PAP animal feed ban 27 Mar 2013 News









Tuesday, March 5, 2013


Use of Materials Derived From Cattle in Human Food and Cosmetics; Reopening of the Comment Period FDA-2004-N-0188-0051 (TSS SUBMISSION)


FDA believes current regulation protects the public from BSE but reopens comment period due to new studies









Thursday, February 14, 2013


The Many Faces of Mad Cow Disease Bovine Spongiform Encephalopathy BSE and TSE prion disease








BSE Case Associated with Prion Protein Gene Mutation (g-h-BSEalabama) and VPSPr PRIONPATHY




*** (see mad cow feed in COMMERCE IN ALABAMA...TSS)


BANNED MAD COW FEED IN COMMERCE IN ALABAMA


Date: September 6, 2006 at 7:58 am PST PRODUCT


a) EVSRC Custom dairy feed, Recall # V-130-6;


b) Performance Chick Starter, Recall # V-131-6;


c) Performance Quail Grower, Recall # V-132-6;


d) Performance Pheasant Finisher, Recall # V-133-6.


CODE None RECALLING FIRM/MANUFACTURER Donaldson & Hasenbein/dba J&R Feed Service, Inc., Cullman, AL, by telephone on June 23, 2006 and by letter dated July 19, 2006. Firm initiated recall is complete.


REASON


Dairy and poultry feeds were possibly contaminated with ruminant based protein.


VOLUME OF PRODUCT IN COMMERCE 477.72 tons


DISTRIBUTION AL


______________________________










PRODUCT Bulk custom dairy pre-mixes,


Recall # V-120-6 CODE None RECALLING FIRM/MANUFACTURER Ware Milling Inc., Houston, MS, by telephone on June 23, 2006. Firm initiated recall is complete. REASON Possible contamination of dairy animal feeds with ruminant derived meat and bone meal.


VOLUME OF PRODUCT IN COMMERCE 350 tons


DISTRIBUTION AL and MS


______________________________


PRODUCT


a) Tucker Milling, LLC Tm 32% Sinking Fish Grower, #2680-Pellet, 50 lb. bags, Recall # V-121-6;


b) Tucker Milling, LLC #31120, Game Bird Breeder Pellet, 50 lb. bags, Recall # V-122-6;


c) Tucker Milling, LLC #31232 Game Bird Grower, 50 lb. bags, Recall # V-123-6;


d) Tucker Milling, LLC 31227-Crumble, Game Bird Starter, BMD Medicated, 50 lb bags, Recall # V-124-6;


e) Tucker Milling, LLC #31120, Game Bird Breeder, 50 lb bags, Recall # V-125-6;


f) Tucker Milling, LLC #30230, 30 % Turkey Starter, 50 lb bags, Recall # V-126-6;


g) Tucker Milling, LLC #30116, TM Broiler Finisher, 50 lb bags, Recall # V-127-6


CODE All products manufactured from 02/01/2005 until 06/20/2006 RECALLING FIRM/MANUFACTURER Recalling Firm: Tucker Milling LLC, Guntersville, AL, by telephone and visit on June 20, 2006, and by letter on June 23, 2006. Manufacturer: H. J. Baker and Brothers Inc., Stamford, CT. Firm initiated recall is ongoing.


REASON Poultry and fish feeds which were possibly contaminated with ruminant based protein were not labeled as "Do not feed to ruminants".


VOLUME OF PRODUCT IN COMMERCE 7,541-50 lb bags


DISTRIBUTION AL, GA, MS, and TN


END OF ENFORCEMENT REPORT FOR AUGUST 9, 2006


###








Subject: MAD COW FEED RECALL AL AND FL VOLUME OF PRODUCT IN COMMERCE 125 TONS Products manufactured from 02/01/2005 until 06/06/2006




Date: August 6, 2006 at 6:16 pm PST PRODUCT


a) CO-OP 32% Sinking Catfish, Recall # V-100-6;


b) Performance Sheep Pell W/Decox/A/N, medicated, net wt. 50 lbs, Recall # V-101-6;


c) Pro 40% Swine Conc Meal -- 50 lb, Recall # V-102-6;


d) CO-OP 32% Sinking Catfish Food Medicated, Recall # V-103-6;


e) "Big Jim's" BBB Deer Ration, Big Buck Blend, Recall # V-104-6;


f) CO-OP 40% Hog Supplement Medicated Pelleted, Tylosin 100 grams/ton, 50 lb. bag, Recall # V-105-6;


g) Pig Starter Pell II, 18% W/MCDX Medicated 282020, Carbadox -- 0.0055%, Recall # V-106-6;


h) CO-OP STARTER-GROWER CRUMBLES, Complete Feed for Chickens from Hatch to 20 Weeks, Medicated, Bacitracin Methylene Disalicylate, 25 and 50 Lbs, Recall # V-107-6;


i) CO-OP LAYING PELLETS, Complete Feed for Laying Chickens, Recall # 108-6;


j) CO-OP LAYING CRUMBLES, Recall # V-109-6;


k) CO-OP QUAIL FLIGHT CONDITIONER MEDICATED, net wt 50 Lbs, Recall # V-110-6;


l) CO-OP QUAIL STARTER MEDICATED, Net Wt. 50 Lbs, Recall # V-111-6;


m) CO-OP QUAIL GROWER MEDICATED, 50 Lbs, Recall # V-112-6 CODE


Product manufactured from 02/01/2005 until 06/06/2006


RECALLING FIRM/MANUFACTURER Alabama Farmers Cooperative, Inc., Decatur, AL, by telephone, fax, email and visit on June 9, 2006. FDA initiated recall is complete.


REASON Animal and fish feeds which were possibly contaminated with ruminant based protein not labeled as "Do not feed to ruminants".


VOLUME OF PRODUCT IN COMMERCE 125 tons


DISTRIBUTION AL and FL


END OF ENFORCEMENT REPORT FOR AUGUST 2, 2006


###







MAD COW FEED RECALL USA EQUALS 10,878.06 TONS NATIONWIDE Sun Jul 16, 2006 09:22 71.248.128.67


RECALLS AND FIELD CORRECTIONS: VETERINARY MEDICINE -- CLASS II


______________________________


PRODUCT


a) PRO-LAK, bulk weight, Protein Concentrate for Lactating Dairy Animals, Recall # V-079-6;


b) ProAmino II, FOR PREFRESH AND LACTATING COWS, net weight 50lb (22.6 kg), Recall # V-080-6;


c) PRO-PAK, MARINE & ANIMAL PROTEIN CONCENTRATE FOR USE IN ANIMAL FEED, Recall # V-081-6;


d) Feather Meal, Recall # V-082-6 CODE


a) Bulk


b) None


c) Bulk


d) Bulk


RECALLING FIRM/MANUFACTURER H. J. Baker & Bro., Inc., Albertville, AL, by telephone on June 15, 2006 and by press release on June 16, 2006. Firm initiated recall is ongoing.


REASON


Possible contamination of animal feeds with ruminent derived meat and bone meal.


VOLUME OF PRODUCT IN COMMERCE 10,878.06 tons


DISTRIBUTION Nationwide


END OF ENFORCEMENT REPORT FOR July 12, 2006


###







2007




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







Subject: bovine blood meal was cross-contaminated with prohibited bovine meat and bone meal 1,366,128 lbs. WI, TX, NE, TN, CO, and MN FEBRUARY 7, 2007 PRODUCT


Bulk Darling's 85% Blood Meal, Flash Dried, distributed in totes and in 1-ton bags (for one customer only), Recall # V-012-2007


CODE


Blood meal distributed between 9/7/2006-2/3/2007.


RECALLING FIRM/MANUFACTURER


Darling National LLC, Omaha, NB, by telephone on January 12, 2007. Firm initiated recall is ongoing.


REASON


Some of the exempt bovine blood meal was cross-contaminated with prohibited bovine meat and bone meal that had been manufactured on common equipment and the labeling did not bear the cautionary BSE statement that it should not be fed to ruminants.


VOLUME OF PRODUCT IN COMMERCE


1,366,128 lbs.


DISTRIBUTION


WI, TX, NE, TN, CO, and MN


END OF ENFORCEMENT REPORT FOR FEBRUARY 7, 2007


###









PRODUCT


O-NO-MORE (Formerly ORPHAN-NO-MORE) Calf Claimer Powder, packaged in 11-oz. bottles, For Animal Use Only. Recall # V-043-2007


CODE


A06


RECALLING FIRM/MANUFACTURER


Springer Magrath Co., Mc Cook, NE, by telephone on January 2, 2007, fax dated January 9, 2007, by letters on February 22, 2007, March 12, March 14 and March 21, 2007. Firm initiated recall is ongoing.


REASON


The finished product was manufactured with prohibited bovine blood meal and did not bear the cautionary BSE statement that the product should not be fed to ruminants.


VOLUME OF PRODUCT IN COMMERCE


Approximately 13,255 bottles


DISTRIBUTION


Nationwide


END OF ENFORCEMENT REPORT FOR JUNE 13, 2007 ###









Saturday, August 4, 2012


Final Feed Investigation Summary - California BSE Case - July 2012












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






Saturday, May 26, 2012


Are USDA assurances on mad cow case 'gross oversimplification'?











Monday, March 25, 2013


Minnesota Firm Recalls Bone-In Ribeye That May Contain Specified Risk Materials Recall Release CLASS II RECALL FSIS-RC-024-2013








Saturday, December 15, 2012


Bovine spongiform encephalopathy: the effect of oral exposure dose on attack rate and incubation period in cattle -- an update 5 December 2012














Thursday, April 4, 2013


Variably protease-sensitive prionopathy in the UK: a retrospective review 1991–2008


Brain (2013) 136 (4): 1102-1115. doi: 10.1093/brain/aws366







http://creutzfeldt-jakob-disease.blogspot.com/





TSS
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Wednesday, April 10, 2013

CFIA investigated for muzzling scientists

CFIA investigated for muzzling scientists





Posted Apr. 5th, 2013by Barry WilsonNo Comments





The Canadian Food Inspection Agency is one of seven government departments and agencies that the federal information commissioner is investigating for alleged lack of information transparency.



Commissioner Suzanne Legault received a complaint from the University of Victoria’s Environmental Law Clinic (ELC) and the Ottawa-based advocacy group Democracy Watch that government muzzling of scientists is a violation of the Access to Information Act.



The complaint about the CFIA is based on an incident in Calgary late last year during a briefing on a massive beef recall because of E coli contamination discovered in product from the XL Foods Inc. plant in Brooks, Alta.



“George Da Pont, president of the CFIA, attended a live news conference to discuss the government’s handling of the recall and answer questions,” said the complaint sent to Legault.



“However, the conference was abruptly ended when an assistant of agriculture minister Gerry Ritz interrupted Da Pont mid-sentence and ushered him away from the cameras.”


Meagan Murdoch, the minister’s director of communications at the time, directed Da Pont away from the microphone, abruptly ending a news conference being televised nationally.



Democracy Watch co-ordinator Tyler Sommers said the Calgary incident is the only evidence submitted on CFIA information practices.



“The information commissioner may be working on other information as well, but that is all we submitted,” he said.



The agency issued a statement indicating it is co-operating.



“As Canada’s largest science-based regulator, the CFIA is proud of the contributions we make to the quality of life of Canadians,” it said.



“The CFIA continuously strives to be transparent and accountable in how it does business. We will fully cooperate with whatever we are asked to provide over the course of the investigation.”



Also under investigation are the departments of environment, fisheries and oceans, national defence, natural resources, treasury board and the National Research Council of Canada.



In the request for an investigation, the ELC and Democracy Watch accused the federal government of “systematic efforts to obstruct the right of the media, and through them the Canadian public, to timely access to government scientists. There are few issues more fundamental to democracy than the ability of the public to access scientific information produced by government scientists, information that their tax dollars have paid for.”



It included a 130-page report, Muzzling Civil Servants: A Threat to Democracy.












CENSORSHIP IS A TERRIBLE THING $$$





Canada has had a COVER-UP policy of mad cow disease since about the 17th case OR 18th case of mad cow disease. AFTER THAT, all FOIA request were ignored $$$


THIS proves there is indeed an epidemic of mad cow disease in North America, and it has been covered up for years and years, if not for decades, and it’s getting worse $$$





Thursday, February 10, 2011


TRANSMISSIBLE SPONGIFORM ENCEPHALOPATHY REPORT UPDATE CANADA FEBRUARY 2011 and how to hide mad cow disease in Canada Current as of: 2011-01-31









Thursday, January 17, 2013


Canada, U.S. agree on animal-disease measures to protect trade, while reducing human and animal health protection









Wednesday, August 11, 2010


REPORT ON THE INVESTIGATION OF THE SIXTEENTH CASE OF BOVINE SPONGIFORM ENCEPHALOPATHY (BSE) IN CANADA








Thursday, August 19, 2010


REPORT ON THE INVESTIGATION OF THE SEVENTEENTH CASE OF BOVINE SPONGIFORM ENCEPHALOPATHY (BSE) IN CANADA









Friday, March 4, 2011


Alberta dairy cow found with mad cow disease









Increased Atypical Scrapie Detections


Press reports indicate that increased surveillance is catching what otherwise would have been unreported findings of atypical scrapie in sheep. In 2009, five new cases have been reported in Quebec, Ontario, Alberta, and Saskatchewan. With the exception of Quebec, all cases have been diagnosed as being the atypical form found in older animals. Canada encourages producers to join its voluntary surveillance program in order to gain scrapie-free status. The World Animal Health will not classify Canada as scrapie-free until no new cases are reported for seven years. The Canadian Sheep Federation is calling on the government to fund a wider surveillance program in order to establish the level of prevalence prior to setting an eradication date. Besides long-term testing, industry is calling for a compensation program for farmers who report unusual deaths in their flocks.









another atypical Nor-98 Scrapie case documented in Canada for 2012


Date confirmed Location Animal type infected May 31* Quebec Sheep









Sunday, April 29, 2012


Scrapie confirmed at quarantined sheep farm Canada CFIA









Wednesday, April 4, 2012


20120402 - Breach of quarantine/Violation de la mise en quarantaine of an ongoing Scrapie investigation









Thursday, February 23, 2012


Atypical Scrapie NOR-98 confirmed Alberta Canada sheep January 2012









***




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.









Friday, November 09, 2012


*** Chronic Wasting Disease CWD in cervidae and transmission to other species









Sunday, November 11, 2012


*** Susceptibilities of Nonhuman Primates to Chronic Wasting Disease November 2012









Friday, December 14, 2012


Susceptibility Chronic Wasting Disease (CWD) in wild cervids to Humans 2005 - December 14, 2012










The chances of a person or domestic animal contracting CWD are “extremely remote,” Richards said. The possibility can’t be ruled out, however. “One could look at it like a game of chance,” he explained. “The odds (of infection) increase over time because of repeated exposure. That’s one of the downsides of having CWD in free-ranging herds: We’ve got this infectious agent out there that we can never say never to in terms of (infecting) people and domestic livestock.”










The Canadian Food Inspection Agency has a small laboratory that does some work on CWD, but only for game-ranched animals. It does not participate in monitoring programs.


The disease slowly kills animals, shortening females' reproductive lives, and will eventually cause numbers to drop. If the number of fawns that survive to become reproducing adults drops to less than one per female, "there'll be no stopping it the longer we wait, not that we know how to stop it now," Leighton said.


Research needs to be maintained, and it will take time for current research to translate into new tools for trying to reduce the impact of the disease, he said.


badam@thestarphoenix.com


© Copyright (c) The StarPhoenix









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





"From the data that we do have, it's quite astonishing ... This disease is on the march in a major way," said Ted Leighton, executive director of the Canadian Co-operative Wildlife Health Centre at the University of Saskatchewan college of veterinary medicine.


"This is getting to levels where we can start to be concerned about actual effects on wild deer populations," he said.


In February, a yearling elk found dead in a Nipawin farmyard was the first wild elk in the province to test positive for CWD.


The disease has been tracked among farmed elk populations for a number of years.


Between 13 and 24 per cent of a small sample of wild cervids (deer and elk) tested in the Nipawin area northeast of Prince Albert have been found to have CWD. It has also been found in a moose in Alberta, and experiments have shown it can be transmitted to caribou, though none have been found in the wild.


The disease is caused by little-understood agents, called prions, that attack the brain and cause death.


Prions also cause bovine spongiform encephalopathy, commonly known as mad cow disease in cattle and Creutzfeldt-Jakob disease in humans.


Higher rates of CWD infection are found in cervids along the South Saskatchewan River toward the Alberta border, where it has been studied the most intensively, Leighton said.









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





Tuesday, March 19, 2013


CWD found in wild deer, elk Saskatchewan a slowly spreading epidemic there'll be no stopping it the longer we wait









very disturbing...tss


Wednesday, December 05, 2012


CANADA CWD VOLUNTARY HERD CERTIFICATION PROGRAM 2012-12-05 Accredited Veterinarian's Manual / Manuel du vétérinaire accrédité









Thursday, November 29, 2012




Chronic wasting disease on the Canadian prairies




snip...



This apparent apathy comes at a time when evidence is accumulating that CWD will cause population declines and altered age structures. In the only population of free-ranging deer in Canada being closely monitored for changes in CWD prevalence and survival, we estimate CWD prevalence in adult deer is now approximately 50% and is the main cause of mortality in adult deer. The study area is immediately adjacent to one of the first elk farms to test positive for CWD and it is likely that close to 15 years of infection in this wild population has resulted in ever increasing environmental burdens of prions which is now driving the outbreak. Detailed radio-tracking and motion sensitive photography is showing that mule deer in this area repeatedly and heavily use anthropogenic sites such as leaking grain bins, cattle salt blocks, hay bales, etc. Increased congregation and contamination of these sites with urine, saliva and feces increases the risk of CWD transmission. In essence these wild deer are behaving similar to deer on game farms except their movement isn’t constrained by a fence. The outcome is likely to be the same, extremely high infection rates and drastic population declines. ...



snip...










Because of their close taxonomic relationship and similarities in DNA sequences of the prion protein (PrP) coding region to deer and wapiti, it had been hypothesized that moose (Alces alces shirasi) would be naturally susceptible to infection if sufficient exposure to the CWD agent occurred (Williams, 2005).


A recent experiment using oral exposure to infectious brain tissue in captive moose confirmed that this species is susceptible to CWD (Kreeger et al., 2006). Here, we report a natural case of CWD in a free-ranging moose from north central Colorado.









Anim Genet. 2006 August; 37(4): 425–426. doi: 10.1111/j.1365-2052.2006.01466.x PMCID: PMC1592321


Polymorphisms of the prion protein gene (PRNP) in Alaskan moose (Alces alces gigas)


“Genetic similarities, susceptibility in the laboratory setting and overlapping geographical ranges suggest the lack of a barrier to the transmission of prion disease from mule and white-tailed deer to moose.”












Tuesday, June 26, 2012


Creutzfeldt Jakob Disease Human TSE report update North America, Canada, Mexico, and USDA PRION UNIT as of May 18, 2012


type determination pending Creutzfeldt Jakob Disease (tdpCJD), is on the rise in Canada and the USA










Sunday, December 2, 2012


CANADA 19 cases of mad cow disease SCENARIO 4: ‘WE HAD OUR CHANCE AND WE BLEW IT’










Thursday, February 14, 2013


The Many Faces of Mad Cow Disease Bovine Spongiform Encephalopathy BSE and TSE prion disease










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










16 YEAR OLD SPORADIC FFI ?


Monday, January 14, 2013


Gambetti et al USA Prion Unit change another highly suspect USA mad cow victim to another fake name i.e. sporadic FFI at age 16 CJD Foundation goes along with this BSe








Monday, December 31, 2012


Creutzfeldt Jakob Disease and Human TSE Prion Disease in Washington State, 2006–2011-2012









Tuesday, December 25, 2012


CREUTZFELDT JAKOB TSE PRION DISEASE HUMANS END OF YEAR REVIEW DECEMBER 25, 2012









Tuesday, June 26, 2012


Creutzfeldt Jakob Disease Human TSE report update North America, Canada, Mexico, and USDA PRION UNIT as of May 18, 2012


type determination pending Creutzfeldt Jakob Disease (tdpCJD), is on the rise in Canada and the USA









Sunday, March 31, 2013


Creutzfeldt Jakob Disease CJD worlds youngest documented victim, 11 years old, shall we pray











Thursday, April 4, 2013


Variably protease-sensitive prionopathy in the UK: a retrospective review 1991–2008


Brain (2013) 136 (4): 1102-1115. doi: 10.1093/brain/aws366








IT is of my opinion, that the OIE and the USDA et al, are the soul reason, and responsible parties, for Transmissible Spongiform Encephalopathy TSE prion diseases, including typical and atypical BSE, typical and atypical Scrapie, and all strains of CWD, and human TSE there from, spreading around the globe.





I have lost all confidence of this organization as a regulatory authority on animal disease, and consider it nothing more than a National Trading Brokerage for all strains of animal TSE, just to satisfy there commodity. AS i said before, OIE should hang up there jock strap now, since it appears they will buckle every time a country makes some political hay about trade protocol, commodities and futures. IF they are not going to be science based, they should do everyone a favor and dissolve there organization.





JUST because of low documented human body count with nvCJD and the long incubation periods, the lack of sound science being replaced by political and corporate science in relations with the fact that science has now linked some sporadic CJD with atypical BSE and atypical scrapie, and the very real threat of CWD being zoonosis, I believed the O.I.E. has failed terribly and again, I call for this organization to be dissolved. ...








Tuesday, July 17, 2012



O.I.E. BSE, CWD, SCRAPIE, TSE PRION DISEASE Final Report of the 80th General Session, 20 - 25 May 2012



http://transmissiblespongiformencephalopathy.blogspot.com/2012/07/oie-bse-cwd-scrapie-tse-prion-disease.html







Thursday, December 20, 2012



OIE GROUP RECOMMENDS THAT SCRAPE PRION DISEASE BE DELISTED AND SAME OLD BSe WITH BOVINE MAD COW DISEASE



http://transmissiblespongiformencephalopathy.blogspot.com/2012/12/oie-group-recommends-that-scrape-prion.html






TSS
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Monday, March 25, 2013

Minnesota Firm Recalls Bone-In Ribeye That May Contain Specified Risk Materials Recall Release CLASS II RECALL FSIS-RC-024-2013

Minnesota Firm Recalls Bone-In Ribeye That May Contain Specified Risk Materials



Recall Release



CLASS II RECALL



FSIS-RC-024-2013 HEALTH RISK: LOW




Congressional and Public Affairs Atiya Khan (202) 720-9113




WASHINGTON, March 25, 2013 – Triple J Family Farms, a Buffalo Lake, Minn. establishment, is recalling approximately 15,270 pounds of bone-in ribeye products because the vertebral column may not have been completely removed, which is not compliant with regulations that require the removal of vertebral column in cattle 30 months of age or older, the U.S. Department of Agriculture's Food Safety and Inspection Service (FSIS) announced today.




The products subject to recall are: [View Labels (PDF Only)]




Approx. 40-lb boxes of "BEEF B/I RIB," bearing any of the following case codes: "91-R109H-C," "91-R109H-S," "91-R109H-C-SB," or "91-R109H-S-SB."




The products subject to recall bear the establishment number "EST.17466" inside the USDA mark of inspection. The products were produced and packaged on various dates between Feb. 8, 2013, and March 21, 2013, and were distributed to an FSIS-inspected establishment in New York for further processing and distribution.




The problem was discovered by FSIS during a routine specified risk material (SRM) verification and may have occurred as a result of a recent change in the company's carcass separation practices. Vertebral column is considered a SRM and must be removed from cattle of 30 months of age or older in accordance with FSIS regulations. SRMs are tissues that may contain the infective agent in cattle infected with Bovine Spongiform Encephalopathy (BSE), as well as materials that are closely associated with these potentially infective tissues. Therefore, FSIS prohibits SRMs from use as human food to minimize potential human exposure to the BSE agent. There is no indication that any of the cattle slaughtered displayed any signs of BSE.




FSIS routinely conducts recall effectiveness checks to verify recalling firms notify their customers of the recall and that steps are taken to make certain that the product is no longer available to consumers.




Consumers with questions about the recall should contact the company's HR and Office Manager, Kendra Williams, at (320) 833-2001. Media with questions about the recall should contact the company's QA Manager, Russell Harris, at (320) 833-0107.




Consumers with food safety questions can "Ask Karen," the FSIS virtual representative available 24 hours a day at AskKaren.gov or via smartphone at m.askkaren.gov. "Ask Karen" live chat services are available Monday through Friday from 10 a.m. to 4 p.m. ET. The toll-free USDA Meat and Poultry Hotline 1-888-MPHotline (1-888-674-6854) is available in English and Spanish and can be reached from l0 a.m. to 4 p.m. (Eastern Time) Monday through Friday. Recorded food safety messages are available 24 hours a day. The online Electronic Consumer Complaint Monitoring System can be accessed 24 hours a day at: www.fsis.usda.gov/FSIS_Recalls/ Problems_With_Food_Products/index.asp






#












see labels ;












Saturday, December 15, 2012



*** Bovine spongiform encephalopathy: the effect of oral exposure dose on attack rate and incubation period in cattle -- an update 5 December 2012











Saturday, November 10, 2012


Wisconsin Firm Recalls Beef Tongues That May Contain Specified Risk Materials Nov 9, 2012 WI Firm Recalls Beef Tongues










Saturday, July 23, 2011



CATTLE HEADS WITH TONSILS, BEEF TONGUES, SPINAL CORD, SPECIFIED RISK MATERIALS (SRM's) AND PRIONS, AKA MAD COW DISEASE










Sunday, October 18, 2009


Wisconsin Firm Recalls Beef Tongues That Contain Prohibited Materials SRM WASHINGTON, October 17, 2009










Thursday, October 15, 2009


Nebraska Firm Recalls Beef Tongues That Contain Prohibited Materials SRM WASHINGTON, Oct 15, 2009








Thursday, June 26, 2008


Texas Firm Recalls Cattle Heads That Contain Prohibited Materials









Tuesday, July 1, 2008



Missouri Firm Recalls Cattle Heads That Contain Prohibited Materials SRMs










Friday, August 8, 2008


Texas Firm Recalls Cattle Heads That Contain Prohibited Materials SRMs 941,271 pounds with tonsils not completely removed










Saturday, April 5, 2008


SRM MAD COW RECALL 406 THOUSAND POUNDS CATTLE HEADS WITH TONSILS KANSAS










Wednesday, April 30, 2008


Consumption of beef tongue: Human BSE risk associated with exposure to lymphoid tissue in bovine tongue in consideration of new research findings










Wednesday, April 30, 2008


Consumption of beef tongue: Human BSE risk associated with exposure to lymphoid tissue in bovine tongue in consideration of new research findings










Friday, October 15, 2010


BSE infectivity in the absence of detectable PrPSc accumulation in the tongue and nasal mucosa of terminally diseased cattle









SPECIFIED RISK MATERIALS SRMs












2007




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











Saturday, August 4, 2012


Final Feed Investigation Summary - California BSE Case - July 2012









Saturday, August 4, 2012


Update from APHIS Regarding Release of the Final Report on the BSE Epidemiological Investigation











2012-2013








Wednesday, March 20, 2013




GAO-13-244, Mar 18, 2013 Dietary Supplements FDA May Have Opportunities to Expand Its Use of Reported Health Problems to Oversee Product



From: Terry S. Singeltary Sr.



Sent: Tuesday, March 19, 2013 2:46 PM






Cc: siggerudk@gao.gov ; youngc1@gao.gov ; oighotline@gao.gov


















Wednesday, February 20, 2013



World Organization for Animal Health Recommends United States' BSE Risk Status Be Upgraded



Statement from Agriculture Secretary Tom Vilsack:















Thursday, February 14, 2013



The Many Faces of Mad Cow Disease Bovine Spongiform Encephalopathy BSE and TSE prion disease












look at the table and you'll see that as little as 1 mg (or 0.001 gm) caused 7% (1 of 14) of the cows to come down with BSE;




Risk of oral infection with bovine spongiform encephalopathy agent in primates





Corinne Ida Lasmézas, Emmanuel Comoy, Stephen Hawkins, Christian Herzog, Franck Mouthon, Timm Konold, Frédéric Auvré, Evelyne Correia, Nathalie Lescoutra-Etchegaray, Nicole Salès, Gerald Wells, Paul Brown, Jean-Philippe Deslys Summary The uncertain extent of human exposure to bovine spongiform encephalopathy (BSE)--which can lead to variant Creutzfeldt-Jakob disease (vCJD)--is compounded by incomplete knowledge about the efficiency of oral infection and the magnitude of any bovine-to-human biological barrier to transmission. We therefore investigated oral transmission of BSE to non-human primates. We gave two macaques a 5 g oral dose of brain homogenate from a BSE-infected cow. One macaque developed vCJD-like neurological disease 60 months after exposure, whereas the other remained free of disease at 76 months. On the basis of these findings and data from other studies, we made a preliminary estimate of the food exposure risk for man, which provides additional assurance that existing public health measures can prevent transmission of BSE to man.



snip...



BSE bovine brain inoculum



100 g 10 g 5 g 1 g 100 mg 10 mg 1 mg 0·1 mg 0·01 mg



Primate (oral route)* 1/2 (50%)



Cattle (oral route)* 10/10 (100%) 7/9 (78%) 7/10 (70%) 3/15 (20%) 1/15 (7%) 1/15 (7%)



RIII mice (ic ip route)* 17/18 (94%) 15/17 (88%) 1/14 (7%)



PrPres biochemical detection



The comparison is made on the basis of calibration of the bovine inoculum used in our study with primates against a bovine brain inoculum with a similar PrPres concentration that was inoculated into mice and cattle.8 *Data are number of animals positive/number of animals surviving at the time of clinical onset of disease in the first positive animal (%). The accuracy of bioassays is generally judged to be about plus or minus 1 log. ic ip=intracerebral and intraperitoneal.


Table 1: Comparison of transmission rates in primates and cattle infected orally with similar BSE brain inocula




Published online January 27, 2005










Experimental BSE Infection of Non-human Primates: Efficacy of the Oral Route




Holznagel, E1; Yutzy, B1; Deslys, J-P2; Lasmézas, C2; Pocchiari, M3; Ingrosso, L3; Bierke, P4; Schulz-Schaeffer, W5; Motzkus, D6; Hunsmann, G6; Löwer, J1 1Paul-Ehrlich-Institut, Germany; 2Commissariat à l´Energie Atomique, France; 3Instituto Superiore di Sanità, Italy; 4Swedish Institute for Infectious Disease control, Sweden; 5Georg August University, Germany; 6German Primate Center, Germany



Background: In 2001, a study was initiated in primates to assess the risk for humans to contract BSE through contaminated food. For this purpose, BSE brain was titrated in cynomolgus monkeys.



Aims: The primary objective is the determination of the minimal infectious dose (MID50) for oral exposure to BSE in a simian model, and, by in doing this, to assess the risk for humans. Secondly, we aimed at examining the course of the disease to identify possible biomarkers.



Methods: Groups with six monkeys each were orally dosed with lowering amounts of BSE brain: 16g, 5g, 0.5g, 0.05g, and 0.005g. In a second titration study, animals were intracerebrally (i.c.) dosed (50, 5, 0.5, 0.05, and 0.005 mg).



Results: In an ongoing study, a considerable number of high-dosed macaques already developed simian vCJD upon oral or intracerebral exposure or are at the onset of the clinical phase. However, there are differences in the clinical course between orally and intracerebrally infected animals that may influence the detection of biomarkers.



Conclusions: Simian vCJD can be easily triggered in cynomolgus monkeys on the oral route using less than 5 g BSE brain homogenate. The difference in the incubation period between 5 g oral and 5 mg i.c. is only 1 year (5 years versus 4 years). However, there are rapid progressors among orally dosed monkeys that develop simian vCJD as fast as intracerebrally inoculated animals.



The work referenced was performed in partial fulfilment of the study “BSE in primates“ supported by the EU (QLK1-2002-01096).










Simian vCJD can be easily triggered in cynomolgus monkeys on the oral route using less than 5 g BSE brain homogenate.









It is clear that the designing scientists must also have shared Mr Bradleys surprise at the results because all the dose levels right down to 1 gram triggered infection.









it is clear that the designing scientists must have also shared Mr Bradleyâs surprise at the results because all the dose levels right down to 1 gram triggered infection.













Prion 7:2, 99–108; March/April 2013; © 2013 Landes Bioscience


mini-Rev iew Mini-REVIEW


A closer look at prion strains




Characterization and important implications




Laura Solforosi,†,* Michela Milani,† Nicasio Mancini, Massimo Clementi and Roberto Burioni


Laboratory of Microbiology and Virology; University Vita-Salute San Raffaele; Milan, Italy


†These authors contributed equally to this work.


Keywords: cellular prion protein (PrPC), scrapie prion protein (PrPSc), transmissible spongiform encephalopathies (TSEs), prion strains, strain mutation, variant Creutzfeldt-Jakob disease, sporadic Creutzfeldt-Jakob disease


Abbreviations: PrPC, cellular prion protein; PrPSc, scrapie prion protein; TSEs, transmissible spongiform encephalopathies; TME, transmissible mink encephalopathy; CJD, Creutzfeldt-Jakob disease; sCJD, sporadic CJD; vCJD, variant CJD; FFI, fatal familial insomnia; BSE, bovine spongiform encephalopathy; CWD, chronic wasting disease; PK, proteinase K; SAF, scrapie-associated fibrils; CNS, central nervous system; WB, western blot; PE, phosphatidylethanolamine; sPMCA, serial protein misfolding cyclic amplification; CPA, cell panel assay




Prions are infectious proteins that are responsible for transmissible spongiform encephalopathies (TSEs) and consist primarily of scrapie prion protein (PrPSc), a pathogenic isoform of the host-encoded cellular prion protein (PrPC). The absence of nucleic acids as essential components of the infectious prions is the most striking feature associated to these diseases. Additionally, different prion strains have been isolated from animal diseases despite the lack of DNA or RNA molecules. Mounting evidence suggests that prion-strain-specific features segregate with different PrPSc conformational and aggregation states.



Strains are of practical relevance in prion diseases as they can drastically differ in many aspects, such as incubation period, PrPSc biochemical profile (e.g., electrophoretic mobility and glycoform ratio) and distribution of brain lesions. Importantly, such different features are maintained after inoculation of a prion strain into genetically identical hosts and are relatively stable across serial passages.



This review focuses on the characterization of prion strains and on the wide range of important implications that the study of prion strains involves.



Introduction



Transmissible spongiform encephalopathies (TSEs) or prion diseases, such as Creutzfeldt-Jakob disease (CJD) in human, bovine spongiform encephalopathy (BSE) in cattle, chronic wasting disease (CWD) in cervids and scrapie in sheep, are a group of fatal neurodegenerative disorders. The major neuropathological hallmarks of TSEs are extensive spongiosis, neuronal cell loss in the central nervous system, gliosis,1 and deposition of amyloid plaques.2



*Correspondence to: Laura Solforosi; Email: solforosi.laura@hsr.it Submitted: 08/13/12; Revised: 12/20/12; Accepted: 01/03/13 http://dx.doi.org/10.4161/pri.23490





Prions are infectious proteins that are responsible for transmissible spongiform encephalopathies (TSEs) and consist primarily of scrapie prion protein (PrPSc), a pathogenic isoform of the host-encoded cellular prion protein (PrPC). The absence of nucleic acids as essential components of the infectious prions is the most striking feature associated to these diseases. Additionally, different prion strains have been isolated from animal diseases despite the lack of DNA or RNA molecules. Mounting evidence suggests that prion-strain-specific features segregate with different PrPSc conformational and aggregation states.





Strains are of practical relevance in prion diseases as they can drastically differ in many aspects, such as incubation period, PrPSc biochemical profile (e.g., electrophoretic mobility and glycoform ratio) and distribution of brain lesions. Importantly, such different features are maintained after inoculation of a prion strain into genetically identical hosts and are relatively stable across serial passages.





This review focuses on the characterization of prion strains and on the wide range of important implications that the study of prion strains involves. ...





snip...





This classification arises from the hypothesis that if the polymorphism 129 can modulate the phenotype of the familial prion diseases (fCJD and FFI, as explained earlier in this review), then probably it can modulate also that of sporadic prion diseases, justifying their heterogeneity. According to this hypothesis, the cases affected by sCJD were divided into six groups according to the genotype of the polymorphism in position 129 and the type of PrPSc. Then, the phenotypes of every group were analyzed to evaluate the homogeneity within every group. The results have permitted a molecular sub-classification of the sCJD.90,91 However, this classification seems not to be sufficient to explain the complexity of the sporadic form of CJD. In fact, in some molecular subtypes, additional variants have been reported, such as MM or VV patients with amyloid plaques, which are absent in the majority of patients with these genotypes.44 Moreover, among patients belonging to the same subgroup, important phenotypic differences can be found, such as, for instance, the extent of neuronal loss or PrPSc deposition differences.92





Even at the biochemical level the complexity is higher: indeed, aside from the migratory differences of the PrPSc of types 1 and 2, there are other properties that could be important during the propagation of the strain, like the presence of other fragments derived from differential cleavage at the C- and N-terminus of the protein, which probably coincide with the presence of other forms of PrPSc with different resistance to PK digestion.44 All these molecular classifications are based upon the principle that in all CNS districts the type of PrPSc is the same, but there are pieces of evidence pointing to the fact that different types of PrPSc can be found in different brain areas.64,93 The first evidence of the presence of more than one form of PrPSc in the brain of a sCJD patient was reported by Puoti in 1999.94 These different types of PrPSc can be found to coexist in the same brain region or they can infect distinct districts. Such co-infection influences the vacuolization and the amyloid aggregates formation.95 Even the ratio between the different glycoforms is determined in a regionspecific manner according to the type of PrPSc (1 or 2) and the genotype of codon 129.





The high degree of phenotypic heterogeneity characterizing sCJD90 can lead to the conclusion that transmission studies will probably identify a broad panel of different prions with a great divergence between strains. However, quite surprisingly, many of the recent studies focusing on the characterization of sCJD subtypes have shown that there is a strong tendency to converge to a limited number of strains. This aspect can find an explanation considering the selection conditions, already described in this review, mediated by the environment in which the prion replicates and by the differences in the amino acid sequence of the PrPC. In particular, studies with bank voles96 and mice97 lead to results that support the idea that there are two principal strains responsible of the sCJD, M1 and V2, and two potential strains, M2 and V1, which need further studies to be confirmed.





Different is the case of vCJD. vCJD has been observed in 12 different countries, but in every registered case the same clinical and pathological characteristics have been found.39 In particular, the PrPSc responsible of the vCJD shows a peculiar WB profile, with the unglycosylated form of the protease-resistant PrPSc of 19 kDa (type 2) and a higher representation of the diglycosilated PrPSc (PrPSc 2B) compared with sCJD.39 Nevertheless, using specific antibodies against type 1 PrPSc, a small amount of PrPSc type 1 with a high percentage of diglycosilated form can be detected in association with PrPSc 2B.98 The 2B type is a useful marker for identifying the replication of BSE prions also in other species, including non-human primates.99 In addition, unlike sporadic and genetic CJD, in vCJD the same biological marker (2B type) has been found in all the analyzed brain areas.100 This strong biochemical and pathological homogeneity is in agreement with the hypothesis of the existence of a unique strain. However, unexpectedly, typization experiments of the strains in different transgenic models have given divergent results. In one of these studies, in a context of homotropic transmission, transgenic mice expressing high levels of human PrPC-M129 were inoculated with vCJD isolates coming from France and from the UK.101 All of the French isolates propagated as vCJD, with abundant amyloid plaques and presence of PrPSc 2B.102 Instead, the isolates from the UK led to the propagation of either vCJD or sCJD.103 In particular, the incubation time was shorter and the lesion profile was different compared with the one obtained with the propagation of the classical vCJD strain. Moreover, early replication of the typical agent of the vCJD in lymphoid tissues was detected, indicating that both strains were present in the inoculum.





This new strain with phenotypical features that were similar to sCJD was found to be of type 1 and the transmission in transgenic mice expressing the bovine PrPC failed, unlike the vCJD classical strain (Type 2B).26 The idea that the infection of vCJD contains a minor component of sCJD prions is supported by many pieces of evidence such as the presence of this prion strain at the first passage or the persistence of both types of PrPSc through serial passages in mice.98 In conclusion, although vCJD is one of the most standardized phenotypes among the prion human diseases characterized by a typical form of PrPSc, the transmission studies of vCJD have shown the great potential of divergence of prions, contrary to the results obtained from the studies of sCJD. This data challenge our ability to recognize the pathologies that can derive from the divergence of the BSE strains when they infect humans, both at the pathological and at the biochemical level.







Conclusion







The discovery of prions has led to new interpretations of the pathogenetic mechanism of protein misfolding diseases. Indeed, the common thought was that a protein misfolding disease could only be caused by a mutation in the primary sequence of an endogenous protein, but the discovery of prions changed this view. In fact, it was demonstrated that a seed of misfolded protein can arise from an exogenous infectious protein, which is able to act as a template or as a catalyst for the formation of new aberrant protein.5,6 Importantly, new evidence shows how processes similar to those described for prions could be implicated in the propagation of misfolded proteins of other neurodegenerative pathologies like Alzheimer disease, Parkinson disease, Huntington disease and amyotrophic lateral sclerosis.104,105





Certainly, one of the most puzzling aspects in the prion field is the existence of different strains of an infectious protein. Nevertheless, such diversity can be accommodated within the protein-only hypothesis, as several robust pieces of experimental evidence indicate that strain-specificity is encoded at the level of the different conformations that the pathogenic protein can adopt. The identification of factors and mechanisms influencing the generation of new prion strains or the selection, from a conformationally heterogeneous PrPSc population, of the most suitable prion conformation in a specific environment, represents an important milestone toward the understanding of the mechanisms of prion strain diversity, which can have fundamental clinical and therapeutic implications. Although considerable advances have been made in the understanding of the phenomenon of prion strains, many pieces of information are still missing, foremost among them the definitive evidence for the structural nature of the differences between prion strains.























Thursday, February 21, 2013


National Prion Disease Pathology Surveillance Center Cases Examined January 16, 2013










16 YEAR OLD SPORADIC FFI ?






Monday, January 14, 2013


Gambetti et al USA Prion Unit change another highly suspect USA mad cow victim to another fake name i.e. sporadic FFI at age 16 CJD Foundation goes along with this BSe











Monday, December 31, 2012


Creutzfeldt Jakob Disease and Human TSE Prion Disease in Washington State, 2006–2011-2012











Tuesday, December 25, 2012


CREUTZFELDT JAKOB TSE PRION DISEASE HUMANS END OF YEAR REVIEW DECEMBER 25, 2012











Tuesday, June 26, 2012


Creutzfeldt Jakob Disease Human TSE report update North America, Canada, Mexico, and USDA PRION UNIT as of May 18, 2012


type determination pending Creutzfeldt Jakob Disease (tdpCJD), is on the rise in Canada and the USA











Wednesday, June 13, 2012


MEXICO IS UNDER or MIS DIAGNOSING CREUTZFELDT JAKOB DISEASE AND OTHER PRION DISEASE SOME WITH POSSIBLE nvCJD











*** The discovery of previously unrecognized prion diseases in both humans and animals (i.e., Nor98 in small ruminants) demonstrates that the range of prion diseases might be wider than expected and raises crucial questions about the epidemiology and strain properties of these new forms. We are investigating this latter issue by molecular and biological comparison of VPSPr, GSS and Nor98.






VARIABLY PROTEASE-SENSITVE PRIONOPATHY IS TRANSMISSIBLE ...price of prion poker goes up again $




OR-10: Variably protease-sensitive prionopathy is transmissible in bank voles




Romolo Nonno,1 Michele Di Bari,1 Laura Pirisinu,1 Claudia D’Agostino,1 Stefano Marcon,1 Geraldina Riccardi,1 Gabriele Vaccari,1 Piero Parchi,2 Wenquan Zou,3 Pierluigi Gambetti,3 Umberto Agrimi1 1Istituto Superiore di Sanità; Rome, Italy; 2Dipartimento di Scienze Neurologiche, Università di Bologna; Bologna, Italy; 3Case Western Reserve University; Cleveland, OH USA




Background. Variably protease-sensitive prionopathy (VPSPr) is a recently described “sporadic”neurodegenerative disease involving prion protein aggregation, which has clinical similarities with non-Alzheimer dementias, such as fronto-temporal dementia. Currently, 30 cases of VPSPr have been reported in Europe and USA, of which 19 cases were homozygous for valine at codon 129 of the prion protein (VV), 8 were MV and 3 were MM. A distinctive feature of VPSPr is the electrophoretic pattern of PrPSc after digestion with proteinase K (PK). After PK-treatment, PrP from VPSPr forms a ladder-like electrophoretic pattern similar to that described in GSS cases. The clinical and pathological features of VPSPr raised the question of the correct classification of VPSPr among prion diseases or other forms of neurodegenerative disorders. Here we report preliminary data on the transmissibility and pathological features of VPSPr cases in bank voles.




Materials and Methods. Seven VPSPr cases were inoculated in two genetic lines of bank voles, carrying either methionine or isoleucine at codon 109 of the prion protein (named BvM109 and BvI109, respectively). Among the VPSPr cases selected, 2 were VV at PrP codon 129, 3 were MV and 2 were MM. Clinical diagnosis in voles was confirmed by brain pathological assessment and western blot for PK-resistant PrPSc (PrPres) with mAbs SAF32, SAF84, 12B2 and 9A2.




Results. To date, 2 VPSPr cases (1 MV and 1 MM) gave positive transmission in BvM109. Overall, 3 voles were positive with survival time between 290 and 588 d post inoculation (d.p.i.). All positive voles accumulated PrPres in the form of the typical PrP27–30, which was indistinguishable to that previously observed in BvM109 inoculated with sCJDMM1 cases.




In BvI109, 3 VPSPr cases (2 VV and 1 MM) showed positive transmission until now. Overall, 5 voles were positive with survival time between 281 and 596 d.p.i.. In contrast to what observed in BvM109, all BvI109 showed a GSS-like PrPSc electrophoretic pattern, characterized by low molecular weight PrPres. These PrPres fragments were positive with mAb 9A2 and 12B2, while being negative with SAF32 and SAF84, suggesting that they are cleaved at both the C-terminus and the N-terminus. Second passages are in progress from these first successful transmissions.




Conclusions. Preliminary results from transmission studies in bank voles strongly support the notion that VPSPr is a transmissible prion disease. Interestingly, VPSPr undergoes divergent evolution in the two genetic lines of voles, with sCJD-like features in BvM109 and GSS-like properties in BvI109.




The discovery of previously unrecognized prion diseases in both humans and animals (i.e., Nor98 in small ruminants) demonstrates that the range of prion diseases might be wider than expected and raises crucial questions about the epidemiology and strain properties of these new forms. We are investigating this latter issue by molecular and biological comparison of VPSPr, GSS and Nor98.












Wednesday, March 28, 2012


VARIABLY PROTEASE-SENSITVE PRIONOPATHY IS TRANSMISSIBLE, price of prion poker goes up again $










*** The discovery of previously unrecognized prion diseases in both humans and animals (i.e., Nor98 in small ruminants) demonstrates that the range of prion diseases might be wider than expected and raises crucial questions about the epidemiology and strain properties of these new forms. We are investigating this latter issue by molecular and biological comparison of VPSPr, GSS and Nor98.






*** atypical Nor-98 Scrapie has spread from coast to coast in the USA 2012



NIAA Annual Conference April 11-14, 2011



San Antonio, Texas











TSS







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