Sunday, December 28, 2008

MAD COW DISEASE USA DECEMBER 28, 2008 an 8 year review of a failed and flawed policy

MAD COW DISEASE USA DECEMBER 28, 2008 an 8 year review of a failed and flawed policy


Greetings,

I thought a quick review of the Bush's terribly flawed and failed mad cow disease policy, from the illegal feeding of literally millions and millions of pounds of highly suspect, and banned mad feed, to the failed BSE surveillance program, all of which exposed, needlessly, millions of people to the mad cow agent i.e. Transmissible Spongiform Encephalopathy. ...

Parentage-based DNA traceback in beef and dairy cattle 2008



http://www.ars.usda.gov/sp2UserFiles/Place/54380570/HeatonPublications/HeatonParentage-Traceback2008o.pdf




48 hour traceback for BSE mad cow disease in the USA ???

NOT in your lifetime !


8 YEARS IN REVIEW OF THE MAD COW DEBACLE IN THE USA ;


FOR IMMEDIATE RELEASE Statement May 4, 2004 Media Inquiries: 301-827-6242 Consumer Inquiries: 888-INFO-FDA

Statement on Texas Cow With Central Nervous System Symptoms On Friday, April 30 th , the Food and Drug Administration learned that a cow with central nervous system symptoms had been killed and shipped to a processor for rendering into animal protein for use in animal feed.

FDA, which is responsible for the safety of animal feed, immediately began an investigation. On Friday and throughout the weekend, FDA investigators inspected the slaughterhouse, the rendering facility, the farm where the animal came from, and the processor that initially received the cow from the slaughterhouse.

FDA's investigation showed that the animal in question had already been rendered into "meat and bone meal" (a type of protein animal feed). Over the weekend FDA was able to track down all the implicated material. That material is being held by the firm, which is cooperating fully with FDA.

Cattle with central nervous system symptoms are of particular interest because cattle with bovine spongiform encephalopathy or BSE, also known as "mad cow disease," can exhibit such symptoms. In this case, there is no way now to test for BSE. But even if the cow had BSE, FDA's animal feed rule would prohibit the feeding of its rendered protein to other ruminant animals (e.g., cows, goats, sheep, bison).

FDA is sending a letter to the firm summarizing its findings and informing the firm that FDA will not object to use of this material in swine feed only. If it is not used in swine feed, this material will be destroyed. Pigs have been shown not to be susceptible to BSE. If the firm agrees to use the material for swine feed only, FDA will track the material all the way through the supply chain from the processor to the farm to ensure that the feed is properly monitored and used only as feed for pigs.

To protect the U.S. against BSE, FDA works to keep certain mammalian protein out of animal feed for cattle and other ruminant animals. FDA established its animal feed rule in 1997 after the BSE epidemic in the U.K. showed that the disease spreads by feeding infected ruminant protein to cattle.

Under the current regulation, the material from this Texas cow is not allowed in feed for cattle or other ruminant animals. FDA's action specifying that the material go only into swine feed means also that it will not be fed to poultry.

FDA is committed to protecting the U.S. from BSE and collaborates closely with the U.S. Department of Agriculture on all BSE issues. The animal feed rule provides crucial protection against the spread of BSE, but it is only one of several such firewalls. FDA will soon be improving the animal feed rule, to make this strong system even stronger.

####

http://www.fda.gov/bbs/topics/news/2004/new01061.html


http://oversight.house.gov/documents/20040607142914-86912.pdf


Release No. 0336.05 Contact: USDA Jim Rogers 202-690-4755 FDA Rae Jones 301-827- 6242

U.S. Department of Agriculture (USDA) Food and Drug Administration (FDA)

Investigation Results of Texas Cow That Tested Positive for Bovine Spongiform Encephalopathy (BSE) Aug. 30, 2005

snip...

During the course of the investigation, USDA removed and tested a total of 67 animals of interest from the farm where the index animal's herd originated. All of these animals tested negative for BSE. 200 adult animals of interest were determined to have left the index farm. Of these 200, APHIS officials determined that 143 had gone to slaughter, two were found alive (one was determined not to be of interest because of its age and the other tested negative), 34 are presumed dead, one is known dead and 20 have been classified as untraceable. In addition to the adult animals, APHIS was looking for two calves born to the index animal. Due to record keeping and identification issues, APHIS had to trace 213 calves. Of these 213 calves, 208 entered feeding and slaughter channels, four are presumed to have entered feeding and slaughter channels and one calf was untraceable.

snip...


http://www.usda.gov/wps/portal/usdahome?contentidonly=true&contentid=2005/08/0336.xml



USDA orders silence on mad cow in Texas

By Steve Mitchell United Press International Published 5/11/2004 10:16 PM

WASHINGTON, May 11 (UPI) -- The U.S. Department of Agriculture has issued an order instructing its inspectors in Texas, where federal madcow disease testing policies recently were violated, not to talk about the cattle disorder with outside parties, United Press International has learned.

The order, sent May 6 by e-mail from the USDA's Dallas district office,was issued in the wake of the April 27 case at Lone Star Beef in San Angelo, in which a cow displaying signs of a brain disorder was not tested for mad cow disease despite a federal policy to screen all such animals.

The deadly illness also is known as bovine spongiform encephalopathy.

Both the USDA and its Inspector General -- amid allegations that an offsite supervisor overruled the opinion of the inspectors on site and made the final decision not to test the animal -- have opened up investigations to determine why agency policy was violated.

The order, which was obtained by UPI, was issued by Ijaz Qazi, circuit supervisor for the USDA's Food Safety and Inspection Service's Dallas district, which covers the entire state. It reads: "All BSE inquiries MUST be directed to Congressional Public Affairs Phone 202-720-9113 attention Rob Larew OR Steve Khon. This is an urgent message. Any question contact me. Ijaz Qazi."

Although the language might sound innocuous, experienced inspectors familiar with USDA parlance have taken to referring to the notice as a "gag order."

The National Joint Council of Food Inspection Locals -- the national inspectors union -- considers the order a violation of inspectors' freespeech rights and is considering legal action against the USDA for breaching the labor agreement they have with the agency.

Inspectors alleged the order also suggests the agency is concerned about its personnel leaking damaging information about either the Texas case or the USDA's overall mad cow disease surveillance program, which has come under fire since the discovery of an infected cow in Washington state last December.

"Anytime the government suppresses an individual's freedom of speech,that's unconstitutional," Gary Dahl, president of Local 925, the Colorado inspectors union, told UPI.

Stanley Painter, chairman of the National Joint Council, said the USDA has sent out notices in the past stating inspectors cannot talk to reporters.

"It's an intimidation thing," Painter told UPI. Inspectors have the right to talk to anybody about any subject, as long as they clarify they are not speaking on behalf of the USDA and they are not doing it on government time, he said.

USDA spokesman Steven Cohen said he was not familiar with the notice from the Dallas office. He said he would look into it, but did not respond by UPI's publication time. In general, Cohen said, "There's an expectation any statement on behalf of the agency would come from the office of communications (in Washington.)"

Asked if employees could speak freely as long as they clarified that their views did not reflect those of the agency, Cohen said, "We'd rather that agency policy be communicated by those in a position to speak for the agency."

Qazi told UPI the notice was not issued in conjunction with the Texas case and it was routine agency practice that outside inquiries be referred to the Washington office. He said inspectors are free to talk to outside parties, including reporters, and he did not consider the e-mail a violation of the labor agreement with the inspectors.

Painter said the USDA's efforts to keep its employees from talking about mad cow would be better spent "with issues like protecting the consuming public instead of trying to hide things." He added he would "just about bet his last nickel" agency management was attempting to suppress information about the Texas case.

"To keep federal employees from reporting government waste, misuse of appropriations -- those types of things -- that's not a good thing either," Dahl said. "If there is something wrong, let's get it out in the open -- let's get it fixed. We're working for the public, the American consumers. I think they have the right to know this," he said.

"And believe me there's so many indicators saying that the USDA's madcow testing program is broken," Dahl added.

At least one member of Congress, Sen. Tom Harkin, D-Iowa, agrees.

Harkin, a long-time critic of the USDA, sent a letter to Agriculture Secretary Ann Veneman on Monday, saying the Texas incident "calls into question the effectiveness and reliability of USDA's current and proposed surveillance system."

The USDA has proposed testing more than 200,000 cows -- or 10 times its current rate -- in an expanded program scheduled to begin June 1. Harkin wrote in the five-page letter, however, that given the realities of the cattle industry, it is "quite doubtful" the USDA will be able to test that many cows, particularly because it had difficulty finding 20,000 last year.

"We simply cannot tolerate a BSE testing system that fails to give valid answers to critical questions for U.S. consumers and foreign customers,"Harkin said in the letter, which sharply criticizes the agency's failure to address explicitly how its new surveillance program will be implemented.

"We look forward to receiving (Harkin's) letter and having the opportunity to review it and respond to him," USDA spokesman Ed Loyd told UPI. "USDA has acknowledged there was a failure in not testing that cow in Texas for BSE, so we are all working to ensure that does not occur again."

Jim Rogers, a spokesman for USDA's Animal and Plant Health InspectionService, which oversees the agency's mad cow surveillance program, told UPI the agency has tested about 15,500 animals since fiscal year 2004 began, on Oct. 1, 2003. However, the agency has refused to identify the states and facilities from which the cows originated. Rogers said UPI would have to seek that information through the Freedom of Information Act.

The question is central to the USDA's implementation of its expanded surveillance program. Downer cows -- those unable to stand or walk --made up the bulk of the animals the agency tested for mad cow inprevious years, but these were banned from being slaughtered for human consumption in December. This means the agency inspectors no longer can obtain brain samples from these cows at slaughterhouses as they could in the past.

Furthermore, the USDA has not provided any evidence it has worked out agreements with rendering facilities or ranchers, where downers and dead cows are now most likely to be found, to obtain the extra animals for testing.

Loyd said the agency is "working very hard to get animals on the farm that would never show up in a processing facility," and he was "not aware of any issues" that would delay the launch of the new program.

However, he was unable to provide the names or locations of the rendering facilities where the agency will be obtaining cow brains for BSE testing. He said he would look into it but did not return two follow-up phone calls from UPI before publication.

--

Steve Mitchell is UPI's Medical Correspondent. E-mail

mhtml:%7B33B38F65-8D2E-434D-8F9B-8BDCD77D3066%7Dmid://00000008/!x-usc:mailto:sciencemail@upi.com

Copyright © 2001-2004 United Press International

http://www.upi.com/view.cfm?StoryID=20040511-015527-4917r



full text ;


http://madcowtesting.blogspot.com/2007/10/bse-base-mad-cow-testing-texas-usa-and.html



BESIDES the Texas mad cow that sat on the shelf for 7+ months before the Honorable Phyllis Fong of the OIG finally did the end around Johanns et al and finally had Weybridge bring that negative cow back from the dead to finally being a confirmed mad cow (hint, hint, getting MRR implemented first), was this simply another bumbling of BSE protocol, or just same old same old;

Jim Rogers (202) 690-4755

USDA Press Office (202) 720-4623

Statement by Chief Veterinary Medical Officer John Clifford Animal and Plant Health Inspection Service Regarding Non-Definitive BSE Test ResultsJuly 27, 2005

snip...

Our laboratory ran the IHC test on the sample and received non-definitive results that suggest the need for further testing. As we have previously experienced, it is possible for an IHC test to yield differing results depending on the “slice” of tissue that is tested. Therefore, scientists at our laboratory and at Weybridge will run the IHC test on additional “slices” of tissue from this animal to determine whether or not it was infected with BSE. We will announce results as soon as they are compiled, which we expect to occur by next week.

I would note that the sample was taken in April, at which time the protocols allowed for a preservative to be used (protocols changed in June 2005). The sample was not submitted to us until last week, because the veterinarian set aside the sample after preserving it and simply forgot to send it in. On that point, I would like to emphasize that while that time lag is not optimal, it has no implications in terms of the risk to human health. The carcass of this animal was destroyed, therefore there is absolutely no risk to human or animal health from this animal.

snip...


http://www.aphis.usda.gov/lpa/news/2005/07/bsestatement_vs.html



Subject: Statement by Dr. John Clifford Regarding Non-Definitive BSE Test ResultsDate: July 27, 2005 at 12:37 pm PST

Jim Rogers (202) 690-4755Jerry Redding (202) 720-6959

Statement by Dr. John Clifford Regarding Non-Definitive BSE Test Results

Late yesterday, we received non-definitive test results on an animal sampled as part of a voluntary extension of our enhanced BSE surveillance program. USDA is conducting further testing at the National Veterinary Services Laboratories in Ames, Iowa, in consultation with experts from the international reference laboratory in Weybridge, England. We are also sending samples from this animal to the Weybridge laboratory for further testing. It is important to note that this animal poses no threat to our food supply because it did not enter the human food or animal feed chains.

The sample was submitted to us by a private veterinarian. As an extension of our enhanced surveillance program, accredited private veterinarians, who often visit farms in remote areas, collect samples when warranted. The sample in question today was taken from a cow that was at least 12 years of age and experienced complications during calving. The veterinarian treated the sample with a preservative, which readies it for testing using the immunohistochemistry (IHC) test - an internationally recognized confirmatory test for BSE. Neither the rapid screening test nor the Western blot confirmatory test can be conducted on a sample that has been preserved.

Our laboratory ran the IHC test on the sample and received non-definitive results that suggest the need for further testing. As we have previously experienced, it is possible for an IHC test to yield differing results depending on the slice of tissue that is tested. Therefore, scientists at our laboratory and at Weybridge will run the IHC test on additional slices of tissue from this animal to determine whether or not it was infected with BSE. We will announce results as soon as they are compiled, which we expect to occur by next week. I would note that the sample was taken in April, at which time the protocols allowed for a preservative to be used (protocols changed in June 2005). The sample was not submitted to us until last week, because the veterinarian set aside the sample after preserving it and simply forgot to send it in. On that point, I would like to emphasize that while that time lag is not optimal, it has no implications in terms of the risk to human health. The carcass of this animal was destroyed, therefore there is absolutely no risk to human or animal health from this animal.

Regardless of the outcome of the further testing, I want to emphasize that human and animal health in the United States are protected by a system of interlocking safeguards. The most important of these is the ban on specified risk materials from the food supply and the Food and Drug Administrationâ?Ts feed ban. And by any measure, the incidence of BSE in this country is extremely low. Our enhanced surveillance program is designed to provide information about the level of prevalence of BSE in the United States. We are extremely gratified that to date, all sectors of the cattle industry have cooperated in this program by submitting samples from more than 419,000 animals from the highest risk populations. To date, only one animal has tested positive for the disease as part of the surveillance program. These interlocking safeguards continue to protect our food supply.

#

Note to Reporters: USDA news releases, program announcements and media advisories are available on the Internet. Go to the APHIS home page at http://www.aphis.usda.gov/ and click on the â?oNewsâ? button. Also, anyone with an e-mail address can sign up to receive APHIS press releases automatically. Send an e-mail message to
lyris@mdrdlyriss10.aphis.usda.gov

and leave the subject blank. In the message, typesubscribe press_releases.USDA

Newsoc.news@usda.gov

202 720-4623----------------------------------------------------

"The sample was submitted to us by a private veterinarian. As an extension of our enhanced surveillance program, accredited private veterinarians who often visit farms in remote areas collect samples when warranted. The sample in question today was taken from a cow that was at least 12 years of age and experienced complications during calving.

"The veterinarian treated the sample with a preservative which readies it for testing using the immunohistochemistry test, an internationally recognized confirmatory test for BSE.

"Neither the rapid screening test nor the Western blot confirmatory test can be conducted on a sample that has been preserved. Our laboratory ran the IHC test on the sample and received non-definitive results that suggest the need for further testing.

"As we have previously experienced, it is possible for an IHC test to yield differing results, depending on the slice of tissue that is tested. Therefore scientists at our laboratory and at Weybridge will run the IHC test on additional slices of tissue from this animal to determine whether or not it was infected with BSE.

"We will announce results as soon as they are compiled, which we expect to occur by next week.

snip...


http://www.usda.gov/wps/portal/usdahome?contentidonly=true&contentid=2005/07/0280.xml



In Reply to: Re: Statement by Dr. John Clifford Regarding Non-Definitive BSE Test Results posted by TSS on July 27, 2005 at 12:53 pm:

o.k., let me get this right. i am pondering here;-)

all the time this TEXAS positive, positive, (secret) positive, inconclusive, negative, then Weybridge confirmed 2nd BSE documented case (thanks to the Honorable Phyllis Fong), all this time this BSe going on in TEXAS, was plastered all over the news, this guy forgot about that sample, and it just sat up on some shelf wasting away for months, as to be in such bad shape, they now cannot even test it properly.

r i g h t ... like ooops, sorry. ...end

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

The animal died in April, but the veterinarian forgot to send the sample to USDA until this month, Mr. Clifford said. "While that time lag is not optimal, it has no implications in terms of the risk to human health," he said.

IHC tests returned conflicting results on the Texas cow. Use of the preservative means that the other tests commonly done when mad cow is suspected, initial rapid screening and Western blot, can't be performed on this sample, the official said. Mr. Clifford said it's possible to get different results, "depending on the slice of tissue that is tested."

The fatal brain-wasting disease is known medically as bovine spongiform encephalopathy, or BSE. In people, eating tainted meat products has been linked to about 150 deaths from a fatal disorder called variant Creutzfeldt-Jakob disease. Most of the deaths were in the United Kingdom, where there was an outbreak in the 1980s and 1990s.

The U.S. banned Canadian cattle in May 2003 following Canada's first case of mad-cow disease. The U.S. was about to lift the ban in March when U.S. District Judge Richard Cebull in Billings, Mont., granted an injunction to a ranchers' group called R-CALF United Stockgrowers of America. The ranchers had sued to keep the border closed to Canadian cattle, saying the disease presented a risk to the U.S. beef industry as well as to American consumers.

The 9th U.S. Circuit Court of Appeals reversed the injunction earlier this month, allowing cattle shipments from Canada to resume. The lifting of the ban reopens the U.S. to cattle younger than 30 months and expands the list of beef products Canada is allowed to ship to the U.S. Older animals are still banned, because infection levels are believed to increase with age.

Copyright © 2005 Associated Press


http://online.wsj.com/


Audit Report

Animal and Plant Health Inspection Service

Bovine Spongiform Encephalopathy (BSE) Surveillance Program - Phase II

and

Food Safety and Inspection Service

Controls Over BSE Sampling, Specified Risk Materials, and Advanced Meat Recovery Products - Phase III

Report No. 50601-10-KC January 2006

Finding 2 Inherent Challenges in Identifying and Testing High-Risk Cattle Still Remain

Our prior report identified a number of inherent problems in identifying and testing high-risk cattle. We reported that the challenges in identifying the universe of high-risk cattle, as well as the need to design procedures to obtain an appropriate representation of samples, was critical to the success of the BSE surveillance program. The surveillance program was designed to target nonambulatory cattle, cattle showing signs of CNS disease (including cattle testing negative for rabies), cattle showing signs not inconsistent with BSE, and dead cattle. Although APHIS designed procedures to ensure FSIS condemned cattle were sampled and made a concerted effort for outreach to obtain targeted samples, industry practices not considered in the design of the surveillance program reduced assurance that targeted animals were tested for BSE.

USDA/OIG-A/50601-10-KC Page 27

observe these animals ante mortem when possible to assure the animals from the target population are ultimately sampled and the clinical signs evaluated.

snip...


http://www.usda.gov/oig/webdocs/50601-10-KC.pdf


In this context, a word is in order about the US testing program. After the discovery of the first (imported) cow in 2003, the magnitude of testing was much increased, reaching a level of >400,000 tests in 2005 (Figure 4). Neither of the 2 more recently indigenously infected older animals with nonspecific clinical features would have been detected without such testing, and neither would have been identified as atypical without confirmatory Western blots. Despite these facts, surveillance has now been decimated to 40,000 annual tests (USDA news release no. 0255.06, July 20, 2006) and invites the accusation that the United States will never know the true status of its involvement with BSE.

In short, a great deal of further work will need to be done before the phenotypic features and prevalence of atypical BSE are understood. More than a single strain may have been present from the beginning of the epidemic, but this possibility has been overlooked by virtue of the absence of widespread Western blot confirmatory testing of positive screening test results; or these new phenotypes may be found, at least in part, to result from infections at an older age by a typical BSE agent, rather than neonatal infections with new "strains" of BSE. Neither alternative has yet been investigated.


http://www.cdc.gov/ncidod/EID/vol12no12/06-0965.htm


CDC DR. PAUL BROWN TSE EXPERT COMMENTS 2006

The U.S. Department of Agriculture was quick to assure the public earlier this week that the third case of mad cow disease did not pose a risk to them, but what federal officials have not acknowledged is that this latest case indicates the deadly disease has been circulating in U.S. herds for at least a decade.

The second case, which was detected last year in a Texas cow and which USDA officials were reluctant to verify, was approximately 12 years old.

These two cases (the latest was detected in an Alabama cow) present a picture of the disease having been here for 10 years or so, since it is thought that cows usually contract the disease from contaminated feed they consume as calves. The concern is that humans can contract a fatal, incurable, brain-wasting illness from consuming beef products contaminated with the mad cow pathogen.

"The fact the Texas cow showed up fairly clearly implied the existence of other undetected cases," Dr. Paul Brown, former medical director of the National Institutes of Health's Laboratory for Central Nervous System Studies and an expert on mad cow-like diseases, told United Press International. "The question was, 'How many?' and we still can't answer that."

Brown, who is preparing a scientific paper based on the latest two mad cow cases to estimate the maximum number of infected cows that occurred in the United States, said he has "absolutely no confidence in USDA tests before one year ago" because of the agency's reluctance to retest the Texas cow that initially tested positive.

USDA officials finally retested the cow and confirmed it was infected seven months later, but only at the insistence of the agency's inspector general.

"Everything they did on the Texas cow makes everything USDA did before 2005 suspect," Brown said. ...snip...end


http://www.upi.com/


CDC - Bovine Spongiform Encephalopathy and Variant Creutzfeldt ... Dr. Paul Brown is Senior Research Scientist in the Laboratory of Central Nervous System ... Address for correspondence: Paul Brown, Building 36, Room 4A-05, ...


http://www.cdc.gov/ncidod/eid/vol7no1/brown.htm


PAUL BROWN COMMENT TO ME ON THIS ISSUE

Tuesday, September 12, 2006 11:10 AM

"Actually, Terry, I have been critical of the USDA handling of the mad cow issue for some years, and with Linda Detwiler and others sent lengthy detailed critiques and recommendations to both the USDA and the Canadian Food Agency."


http://lists.ifas.ufl.edu/cgi-bin/wa.exe?A2=ind0703&L=sanet-mg&T=0&P=8125


Progress Report from the National Prion Disease Pathology Surveillance Center

An Update from Stephen M. Sergay, MB, BCh & Pierluigi Gambetti, MD

April 3, 2008

The importance to public health in the U.S. of timely diagnosis and monitoring of human prion diseases is unquestionable. Here are some compelling reasons for this:

Prion surveillance in cattle has been reduced by 90% (from about 470,000 to 40,000 in the U.S. in 2007 out of about 35 million cattle slaughtered). Termination of human prion surveillance would therefore remove the second line of surveillance, thereby eliminating prion surveillance in the U.S. entirely. This development would be extremely worrisome in view of recent reports that precautions to limit the spread of the prion infectious agent may not have been followed in some slaughter houses in the U.S. Cattle affected with bovine spongiform encephalopathy (BSE) continue to be discovered in Canada, which has more rigorous BSE surveillance than the U.S. At the same time, Canada imposes few limitations in the trade of potentially prion-infectious cattle with the U.S.

Atypical forms of BSE have emerged which, although rare, appear to be more virulent than the classical BSE that causes vCJD.


http://www.aan.com/news/?event=read&article_id=4397&page=72.45.45


National Veterinary Services Laboratory (NVSL) Immunohistochemistry (IHC) Testing Summary

The BSE enhanced surveillance program involves the use of a rapid screening test, followed by confirmatory testing for any samples that come back \"inconclusive.\" The weekly summary below captures all rapid tests conducted as part of the enhanced surveillance effort. It should be noted that since the enhanced surveillance program began, USDA has also conducted approximately 9,200 routine IHC tests on samples that did not first undergo rapid testing. This was done to ensure that samples inappropriate for the rapid screen test were still tested, and also to monitor and improve upon IHC testing protocols. ...


http://www.aphis.usda.gov/lpa/issues/bse_testing/test_results.html


full text ;


http://usdavskorea.blogspot.com/2008/06/oie-recognition-of-bse-status-of.html


""These 9,200 cases were different because brain tissue samples were preserved with formalin, which makes them suitable for only one type of test--immunohistochemistry, or IHC."

THIS WAS DONE FOR A REASON!

THE IHC test has been proven to be the LEAST LIKELY to detect BSE/TSE in the bovine, and these were probably from the most high risk cattle pool, the ones the USDA et al, SHOULD have been testing. ...TSS

USDA 2003

We have to be careful that we don't get so set in the way we do things that we forget to look for different emerging variations of disease. We've gotten away from collecting the whole brain in our systems. We're using the brain stem and we're looking in only one area. In Norway, they were doing a project and looking at cases of Scrapie, and they found this where they did not find lesions or PRP in the area of the obex. They found it in the cerebellum and the cerebrum. It's a good lesson for us. Ames had to go back and change the procedure for looking at Scrapie samples. In the USDA, we had routinely looked at all the sections of the brain, and then we got away from it. They've recently gone back. Dr. Keller: Tissues are routinely tested, based on which tissue provides an 'official' test result as recognized by APHIS.

Dr. Detwiler: That's on the slaughter. But on the clinical cases, aren't they still asking for the brain? But even on the slaughter, they're looking only at the brainstem. We may be missing certain things if we confine ourselves to one area.

snip.............

Dr. Detwiler: It seems a good idea, but I'm not aware of it. Another important thing to get across to the public is that the negatives do not guarantee absence of infectivity. The animal could be early in the disease and the incubation period. Even sample collection is so important. If you're not collecting the right area of the brain in sheep, or if collecting lymphoreticular tissue, and you don't get a good biopsy, you could miss the area with the PRP in it and come up with a negative test. There's a new, unusual form of Scrapie that's been detected in Norway. We have to be careful that we don't get so set in the way we do things that we forget to look for different emerging variations of disease. We've gotten away from collecting the whole brain in our systems. We're using the brain stem and we're looking in only one area. In Norway, they were doing a project and looking at cases of Scrapie, and they found this where they did not find lesions or PRP in the area of the obex. They found it in the cerebellum and the cerebrum. It's a good lesson for us. Ames had to go back and change the procedure for looking at Scrapie samples. In the USDA, we had routinely looked at all the sections of the brain, and then we got away from it. They've recently gone back.

Dr. Keller: Tissues are routinely tested, based on which tissue provides an 'official' test result as recognized by APHIS .

Dr. Detwiler: That's on the slaughter. But on the clinical cases, aren't they still asking for the brain? But even on the slaughter, they're looking only at the brainstem. We may be missing certain things if we confine ourselves to one area.

snip...

FULL TEXT;

Completely Edited Version PRION ROUNDTABLE

Accomplished this day, Wednesday, December 11, 2003, Denver, Colorado


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

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...

4 USDA OIG SEMIANNUAL REPORT TO CONGRESS FY 2007 1st Half


http://www.usda.gov/oig/webdocs/sarc070619.pdf


full text ;


http://foiamadsheepmadrivervalley.blogspot.com/2008/09/re-foia-of-declaration-of-extraordinary.html


STANLEY PRUSINER NOBEL PEACE PRIZE WINNER ON THE PRION

US AG SEC AND LAYCRAFT

“nothing matters, except beef from Canada under 30 months bone in beef product, that’s ALL THAT MATTERS!”

US SENATOR AND STAN THE MAN SLAM USDA ”DAMNING TESTIMONY”

Senator Michael Machado from California

”USDA does not know what’s going on”. ”USDA is protecting the industry”. ” SHOULD the state of California step in”

Stanley Prusiner

”nobody has ever ask us to comment”

”they don’t want us to comment”

”they never ask”

i tried to see Venemon, after Canadian cow was discovered with BSE. went to see lyle. after talking with him…

absolute ignorance…

then thought i should see Venemon…

it was clear his entire policy was to get cattle boneless beef prods across the border…

nothing else mattered…

his aids confirmed this…

5 times i tried to see Venemon, never worked…

eventually met with carl rove the political…

he is the one that arranged meeting with Venemon…

just trying to give you a sense of the distance…

threat to health public safety…

was never contacted…

yes i believe that prions are bad to eat and you can die from them…END

Dr. Stan bashing Ann Veneman - 3 minutes - Damning testimony


http://maddeer.org/video/embedded/08snip.ram


File Name: USDA DON'T ASK DON'T TELL POLICY 02snip.rpm

DAMNING testimony of consumer consumption of Washington mad cow in California


http://www.maddeer.org/video/embedded/02snip.rm


IN A NUT SHELL ;

(Adopted by the International Committee of the OIE on 23 May 2006)

11. Information published by the OIE is derived from appropriate declarations made by the official Veterinary Services of Member Countries.The OIE is not responsible for inaccurate publication of country disease status based on inaccurate information or changes in epidemiological status or other significant events that were not promptly reported to then Central Bureau............


http://www.oie.int/eng/Session2007/RF2006.pdf


full text ;


http://madcowtesting.blogspot.com/2007/10/bse-base-mad-cow-testing-texas-usa-and.html


http://madcowtesting.blogspot.com/



bought and paid for by your local cattle dealer $$$

IN my opinion the WOAH/OIE is nothing more than a organized bunch of lobbyist for the members Countries in support of there INDUSTRY, bound together as one, with the only purpose of open trade for there precious commodities and futures. Speaking only of BSE, they failed at every corner, and then just said to hell with it, well just trade all strains of TSE globally.

snip...

NOW, ask yourself why not one single mad cow has been documented in the USA since the Honorable Phyllis Fong of the OIG did the end around Johanns, Dehaven et al ??? found two atypical BSE or BASE cases and they flat shut it down i tell you. IF the OIE gives a favorable rating, IF the OIE gives any other rating but the lowest, poorest possible BSE/TSE rating, the OIE will have sealed there fate once and for all, because most of the world knows the truth about the USA and there mad cows. THE OIE will then be able to stand side by side with the USA, and proudly claim to have sold there soul to the devil, all for a buck, commodities and futures, to hell with human health. A 'CONTROLLED' RATING IS EXACTLY what the OIE will get if that is what they classify the USA as a 'CONTROLLED RATING'. IT will be controlled by Johanns, Dehaven, and GW. IT WILL BE RIGGED in other words. but that is nothing new, it's been rigged for years. ...


snip...

SEE FULL TEXT with facts and sources @ ;Wednesday, June 11, 2008


OIE Recognition of the BSE Status of Members RESOLUTION No. XXI (Adopted by the International Committee of the OIE on 27 May 2008)



Attachment to Singeltary comment January 28, 2007 Greetings APHIS, I would kindly like to submit the following to ; BSE; MRR; IMPORTATION OF LIVE BOVINES AND PRODUCTS DERIVED FROM BOVINES [Docket No. APHIS-2006-0041] RIN 0579-AC01 [Federal Register: January 9, 2007 (Volume 72, Number 5)] [Proposed Rules] [Page 1101-1129] From the Federal Register Online via GPO Access [wais.access.gpo.gov] [DOCID:fr09ja07-21]


BSE; MRR; IMPORTATION OF LIVE BOVINES AND PRODUCTS DERIVED FROM BOVINES [Docket No. APHIS-2006-0041] RIN 0579-AC01 Date: January 9, 2007 at 9:08 am PST


http://www.regulations.gov/fdmspublic/component/main?main=DocumentDetail&o=09000064801f3412



Saturday, December 27, 2008

Disappointing decision on U.S. beef petition i.e. FORCE FEEDING KOREANS USDA MAD COW BEEF [Editorial] Disappointing decision on U.S. beef petition


http://usdavskorea.blogspot.com/2008/12/disappointing-decision-on-us-beef.html



GWs BSE MRR policy was based on nothing but lies, exposed millions of people (including school children) to the TSE mad cow agent needlessly, all for a dollar $$$


WHAT about that atypical BSE in the USA ???



Research Project: STUDY OF ATYPICAL BSE Location: Virus and Prion Diseases of Livestock

2008 Annual Report

1a.Objectives (from AD-416) The objective of this cooperative research project with Dr. Maria Caramelli from the Italian BSE Reference Laboratory in Turin, Italy, is to conduct comparative studies with the U.S. bovine spongiform encephalopathy (BSE) isolate and the atypical BSE isolates identified in Italy. The studies will cover the following areas: 1. Evaluation of present diagnostics tools used in the U.S. for the detection of atypical BSE cases. 2. Molecular comparison of the U.S. BSE isolate and other typical BSE isolates with atypical BSE cases. 3. Studies on transmissibility and tissue distribution of atypical BSE isolates in cattle and other species.

1b.Approach (from AD-416) This project will be done as a Specific Cooperative Agreement with the Italian BSE Reference Laboratory, Istituto Zooprofilattico Sperimentale del Piemonte, in Turin, Italy. It is essential for the U.S. BSE surveillance program to analyze the effectiveness of the U.S diagnostic tools for detection of atypical cases of BSE. Molecular comparisons of the U.S. BSE isolate with atypical BSE isolates will provide further characterization of the U.S. BSE isolate. Transmission studies are already underway using brain homogenates from atypical BSE cases into mice, cattle and sheep. It will be critical to see whether the atypical BSE isolates behave similarly to typical BSE isolates in terms of transmissibility and disease pathogenesis. If transmission occurs, tissue distribution comparisons will be made between cattle infected with the atypical BSE isolate and the U.S. BSE isolate. Differences in tissue distribution could require new regulations regarding specific risk material (SRM) removal.

3.Progress Report The aim of the cooperative research project "Study of atypical BSE" led by CEA (Italian Reference Centre for Animal TSE) and USDA is to compare Italian and U.S. Bovine sponigiform encephalopathy (BSE) confirmatory protocols in the detection of classical (C-) and atypical (H- and L-type) BSE cases. In the course of this project samples of Italian C-BSE and Italian L-type BSE (BASE), both frozen and formalin fixed, have been sent to USDA laboratories in Ames, to undergo Western blot and Immunohstochemical (IHC) comparison studies for PrP**Sc detection according to U.S. and Italian methods. In 2007, the comparative study between U.S. and Italian BSE confirmatory protocols was performed. The collaborator sent a scientist to Ames to assist in performing the Italian IHC protocol on the BSE samples chosen for the study. Results obtained showed that the Italian and U.S. IHC procedures were alike in PrP**Sc detection regarding its distribution, deposition pattern and intensity of staining on all the C-, L- and H-type BSE cases considered. In addition, the U.S. protocol evidenced the characteristic presence of plaques in the frontal cortex of the Italian BASE case similar to the Italian protocol. Methods used for monitoring include email, site visits, and periodic written reports. This project addresses NP 103, component 8.


http://www.ars.usda.gov/research/projects/projects.htm?ACCN_NO=408490&showpars=true&fy=2008


Evaluation of the Human Transmission Risk of an Atypical Bovine Spongiform Encephalopathy Prion Strain

Qingzhong Kong,1* Mengjie Zheng,1 Cristina Casalone,2 Liuting Qing,1 Shenghai Huang,1? Bikram Chakraborty,1 Ping Wang,1 Fusong Chen,1 Ignazio Cali,1 Cristiano Corona,2 Francesca Martucci,2 Barbara Iulini,2 Pierluigi Acutis,2 Lan Wang,1 Jingjing Liang,1 Meiling Wang,1 Xinyi Li,1 Salvatore Monaco,3 Gianluigi Zanusso,3 Wen-Quan Zou,1 Maria Caramelli,2 and Pierluigi Gambetti1* Department of Pathology, Case Western Reserve University, Cleveland, Ohio 44106,1 CEA, Istituto Zooprofilattico Sperimentale, 10154 Torino, Italy,2 Department of Neurological and Visual Sciences, University of Verona, 37134 Verona, Italy3 *Corresponding author. Mailing address: Department of Pathology, Case Western Reserve University, Cleveland, OH 44106. Phone for Pierluigi Gambetti: (216) 368-0586. Fax: (216) 368-2546. E-mail:

mhtml:%7B33B38F65-8D2E-434D-8F9B-8BDCD77D3066%7Dmid://00000008/!x-usc:mailto:pxg13@case.edu .

Phone for Qingzhong Kong: (216) 368-1756. Fax: (216) 368-2546. E-mail:

mhtml:%7B33B38F65-8D2E-434D-8F9B-8BDCD77D3066%7Dmid://00000008/!x-usc:mailto:qxk2@case.edu ?Present address: Department of Patient Education and Health Information, Cleveland Clinic Foundation, Cleveland, OH 44195. Received November 30, 2007; Accepted January 16, 2008.

Bovine spongiform encephalopathy (BSE), the prion disease in cattle, was widely believed to be caused by only one strain, BSE-C. BSE-C causes the fatal prion disease named new variant Creutzfeldt-Jacob disease in humans. Two atypical BSE strains, bovine amyloidotic spongiform encephalopathy (BASE, also named BSE-L) and BSE-H, have been discovered in several countries since 2004; their transmissibility and phenotypes in humans are unknown. We investigated the infectivity and human phenotype of BASE strains by inoculating transgenic (Tg) mice expressing the human prion protein with brain homogenates from two BASE strain-infected cattle. Sixty percent of the inoculated Tg mice became infected after 20 to 22 months of incubation, a transmission rate higher than those reported for BSE-C. A quarter of BASE strain-infected Tg mice, but none of the Tg mice infected with prions causing a sporadic human prion disease, showed the presence of pathogenic prion protein isoforms in the spleen, indicating that the BASE prion is intrinsically lymphotropic. The pathological prion protein isoforms in BASE strain-infected humanized Tg mouse brains are different from those from the original cattle BASE or sporadic human prion disease. Minimal brain spongiosis and long incubation times are observed for the BASE strain-infected Tg mice. These results suggest that in humans, the BASE strain is a more virulent BSE strain and likely lymphotropic.

http://www.pubmedcentral.nih.gov/articlerender.fcgi?artid=2268471


Thursday, December 04, 2008 2:37 PM

"we have found that H-BSE can infect humans."

personal communication with Professor Kong. ...TSS



November 25, 2008

Update On Feed Enforcement Activities To Limit The Spread Of BSE

http://madcowfeed.blogspot.com/2008/11/november-2008-update-on-feed.html


"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." ... interesting. ... TSS

Thursday, June 05, 2008

Review on the epidemiology and dynamics of BSE epidemics

Vet. Res. (2008) 39:15 www.vetres.org DOI: 10.1051/vetres:2007053 c INRA, EDP Sciences, 2008 Review article

snip...

And last but not least, similarities of PrPres between Htype BSE and human prion diseases like CJD or GSS have been put forward [10], as well as between L-type BSE and CJD [17]. These findings raise questions about the origin and inter species transmission of these prion diseases that were discovered through the BSE active surveillance.

snip...

Cases of atypical BSE have only been found in countries having implemented large active surveillance programs. As of 1st September 2007, 36 cases (16 H, 20 L) have been described all over the world in cattle: Belgium (1 L) [23], Canada (1 H)15, Denmark (1 L)16, France (8 H, 6 L)17, Germany (1 H, 1 L) [13], Italy (3 L)18, Japan (1 L) [71], Netherlands (1 H, 2 L)19, Poland (1 H, 6 L)20, Sweden (1 H)21, United Kingdom (1 H)22, and USA (2 H)23. Another H-type case has been found in a 19 year old miniature zebu in a zoological park in Switzerland [56]. It is noteworthy that atypical cases have been found in countries that did not experience classical BSE so far, like Sweden, or in which only few cases of classical BSE have been found, like Canada or the USA.

And last but not least, similarities of PrPres between Htype BSE and human prion diseases like CJD or GSS have been put forward [10], as well as between L-type BSE and CJD [17]. These findings raise questions about the origin and inter species transmission of these prion diseases that were discovered through the BSE active surveillance.

full text 18 pages ;


http://www.vetres.org/index.php?option=article&access=standard&Itemid=129&url=/articles/vetres/pdf/2008/04/v07232.pdf


snip...


http://bse-atypical.blogspot.com/2008/06/review-on-epidemiology-and-dynamics-of.html


please see full text ;

Monday, December 22, 2008 [Docket No. FDA–2008–D–0597] Draft Guidance for Industry: Small Entities Compliance Guide for Renderers—Substances Prohibited From Use in Animal Food


http://madcowfeed.blogspot.com/2008/12/docket-no-fda2008d0597-draft-guidance.html


Tuesday, November 11, 2008

Transmission of atypical bovine prions to mice transgenic for human prion protein

DOI: 10.3201/eid1412.080941


http://bse-atypical.blogspot.com/2008/11/transmission-of-atypical-bovine-prions.html


Wednesday, August 20, 2008

Bovine Spongiform Encephalopathy Mad Cow Disease typical and atypical strains, was there a cover-up ?

http://bse-atypical.blogspot.com/2008/08/bovine-spongiform-encephalopathy-mad.html


Tuesday, June 3, 2008

SCRAPIE USA UPDATE JUNE 2008 NOR-98 REPORTED PA

http://nor-98.blogspot.com/2008/06/scrapie-usa-update-june-2008-nor-98.html


SCRAPIE USA

http://scrapie-usa.blogspot.com/


Sunday, September 07, 2008

CWD LIVE TEST, and the political aspects or fallout of live testing for BSE in cattle in the USA

http://chronic-wasting-disease.blogspot.com/2008/09/cwd-live-test-and-political-aspects-or.html


Saturday, October 18, 2008

WYOMING STAR VALLEY MOOSE TESTS POSITIVE FOR CWD

http://chronic-wasting-disease.blogspot.com/2008/10/wyoming-star-valley-moose-tests.html


http://chronic-wasting-disease.blogspot.com/


Friday, December 12, 2008

The prion strain phenomenon: Molecular basis and unprecedented features


http://bse-atypical.blogspot.com/2008/12/prion-strain-phenomenon-molecular-basis.html



Wednesday, December 10, 2008

Evaluation of FSIS Management Controls Over Pre-Slaughter Activities (Audit Report 24601-7-KC) USDA CERTIFIED DEADSTOCK DOWNER COW SCHOOL LUNCH PROGRAM


http://downercattle.blogspot.com/2008/12/evaluation-of-fsis-management-controls.html



Wednesday, August 20, 2008 Bovine Spongiform Encephalopathy Mad Cow Disease typical and atypical strains, was there a cover-up ?


http://bse-atypical.blogspot.com/2008/08/bovine-spongiform-encephalopathy-mad.html


A New Prionopathy OR more of the same old BSe and sporadic CJD


http://creutzfeldt-jakob-disease.blogspot.com/2008/08/new-prionopathy-or-more-of-same-old-bse.html


Communicated by: Terry S. Singeltary Sr.[In submitting these data, Terry S. Singeltary Sr. draws attention to the steady increase in the "type unknown" category, which, according to their definition, comprises cases in which vCJD could be excluded. The total of 26 cases for the current year (2007) is disturbing, possibly symptomatic of the circulation of novel agents. Characterization of these agents should be given a high priority. - Mod.CP]


http://pro-med.blogspot.com/2007/11/proahedr-prion-disease-update-2007-07.html

http://www.promedmail.org/pls/askus/f?p=2400:1001:6833194127530602005::NO::F2400_P1001_BACK_PAGE,F2400_P1001_PUB_MAIL_ID:1010,39963


There is a growing number of human CJD cases, and they were presented last week in San Francisco by Luigi Gambatti(?) from his CJD surveillance collection.He estimates that it may be up to 14 or 15 persons which display selectively SPRPSC and practically no detected RPRPSC proteins.

http://www.fda.gov/ohrms/dockets/ac/06/transcripts/1006-4240t1.htm


http://www.fda.gov/ohrms/dockets/ac/06/transcripts/2006-4240t1.pdf


sporadic Fatal Familial Insomnia

http://sporadicffi.blogspot.com/



THE PATHOLOGICAL PROTEINHardcover, 304 pages plus photos and illustrations. ISBN 0-387-95508-9June 2003BY Philip YamCHAPTER 14 LAYING ODDSAnswering critics like Terry Singeltary, who feels that the U.S. under- counts CJD, Schonberger conceded that the current surveillance system has errors but stated that most of the errors will be confined to the older population.


http://www.thepathologicalprotein.com/



The Lancet Infectious Diseases, Volume 3, Issue 8, Page 463, August 2003

doi:10.1016/S1473-3099(03)00715-1Cite or Link Using DOI

Tracking spongiform encephalopathies in North America

Original Text


Xavier Bosch


“My name is Terry S Singeltary Sr, and I live in Bacliff, Texas. I lost my mom to hvCJD (Heidenhain variant CJD) and have been searching for answers ever since. What I have found is that we have not been told the truth. CWD in deer and elk is a small portion of a much bigger problem.”

49-year-old Singeltary is one of a number of people who have remained largely unsatisfied after being told that a close relative died from a rapidly progressive dementia compatible with spontaneous Creutzfeldt-Jakob disease (CJD). So he decided to gather hundreds of documents on transmissible spongiform encephalopathies (TSE) and realized that if Britons could get variant CJD from bovine spongiform encephalopathy (BSE), Americans might get a similar disorder from chronic wasting disease (CWD)the relative of mad cow disease seen among deer and elk in the USA. Although his feverish search did not lead him to the smoking gun linking CWD to a similar disease in North American people, it did uncover a largely disappointing situation.

Singeltary was greatly demoralized at the few attempts to monitor the occurrence of CJD and CWD in the USA. Only a few states have made CJD reportable. Human and animal TSEs should be reportable nationwide and internationally, he complained in a letter to the Journal of the American Medical Association (JAMA 2003; 285: 733). I hope that the CDC does not continue to expect us to still believe that the 85% plus of all CJD cases which are sporadic are all spontaneous, without route or source.

Until recently, CWD was thought to be confined to the wild in a small region in Colorado. But since early 2002, it has been reported in other areas, including Wisconsin, South Dakota, and the Canadian province of Saskatchewan. Indeed, the occurrence of CWD in states that were not endemic previously increased concern about a widespread outbreak and possible transmission to people and cattle.

To date, experimental studies have proven that the CWD agent can be transmitted to cattle by intracerebral inoculation and that it can cross the mucous membranes of the digestive tract to initiate infection in lymphoid tissue before invasion of the central nervous system. Yet the plausibility of CWD spreading to people has remained elusive.

Part of the problem seems to stem from the US surveillance system. CJD is only reported in those areas known to be endemic foci of CWD. Moreover, US authorities have been criticized for not having performed enough prionic tests in farm deer and elk.

Although in November last year the US Food and Drug Administration issued a directive to state public-health and agriculture officials prohibiting material from CWD-positive animals from being used as an ingredient in feed for any animal species, epidemiological control and research in the USA has been quite different from the situation in the UK and Europe regarding BSE.

Getting data on TSEs in the USA from the government is like pulling teeth, Singeltary argues. You get it when they want you to have it, and only what they want you to have.

Norman Foster, director of the Cognitive Disorders Clinic at the University of Michigan (Ann Arbor, MI, USA), says that current surveillance of prion disease in people in the USA is inadequate to detect whether CWD is occurring in human beings; adding that, the cases that we know about are reassuring, because they do not suggest the appearance of a new variant of CJD in the USA or atypical features in patients that might be exposed to CWD. However, until we establish a system that identifies and analyses a high proportion of suspected prion disease cases we will not know for sure. The USA should develop a system modeled on that established in the UK, he points out.

Ali Samii, a neurologist at Seattle VA Medical Center who recently reported the cases of three hunterstwo of whom were friendswho died from pathologically confirmed CJD, says that at present there are insufficient data to claim transmission of CWD into humans; adding that [only] by asking [the questions of venison consumption and deer/elk hunting] in every case can we collect suspect cases and look into the plausibility of transmission further. Samii argues that by making both doctors and hunters more aware of the possibility of prions spreading through eating venison, doctors treating hunters with dementia can consider a possible prion disease, and doctors treating CJD patients will know to ask whether they ate venison.

CDC spokesman Ermias Belay says that the CDC will not be investigating the [Samii] cases because there is no evidence that the men ate CWD-infected meat. He notes that although the likelihood of CWD jumping the species barrier to infect humans cannot be ruled out 100% and that [we] cannot be 100% sure that CWD does not exist in humans& the data seeking evidence of CWD transmission to humans have been very limited.


Greetings,

>>> he complained in a letter to the Journal of the American Medical Association (JAMA 2003; 285: 733). I hope that the CDC does not continue to expect us to still believe that the 85% plus of all CJD cases which are sporadic are all spontaneous, without route or source.<<< href="mhtml:%7B33B38F65-8D2E-434D-8F9B-8BDCD77D3066%7Dmid://00000008/!x-usc:http://www.thelancet.com/journals/laninf/article/PIIS1473-3099(03)00715-1/fulltext">http://www.thelancet.com/journals/laninf/article/PIIS1473-3099(03)00715-1/fulltext

http://www.thelancet.com/journals/laninf/article/PIIS1473309903007151/%20fulltext

http://www.ncbi.nlm.nih.gov/pubmed/12906010

http://infection.thelancet.com/journal/journal.isa



JOURNAL OF NEUROLOGY

MARCH 26, 2003

RE-Monitoring the occurrence of emerging forms of Creutzfeldt-Jakobdisease in the United States


Email Terry S. Singeltary:

flounder9@verizon.net.

I lost my mother to hvCJD (Heidenhain Variant CJD). I would like to comment on the CDC's attempts to monitor the occurrence of emerging forms of CJD. Asante, Collinge et al [1] have reported that BSE transmission to the 129-methionine genotype can lead to an alternate phenotype that is indistinguishable from type 2 PrPSc, the commonest sporadic CJD. However, CJD and all human TSEs are not reportable nationally. CJD and all human TSEs must be made reportable in every state and internationally. I hope that the CDC does not continue to expect us to still believe that the 85%+ of all CJD cases which are sporadic are all spontaneous, without route/source. We have many TSEs in the USA in both animal and man. CWD in deer/elk is spreading rapidly and CWD does transmit to mink, ferret, cattle, and squirrel monkey by intracerebral inoculation. With the known incubation periods in other TSEs, oral transmission studies of CWD may take much longer. Every victim/family of CJD/TSEs should be asked about route and source of this agent. To prolong this will only spread the agent and needlessly expose others. In light of the findings of Asante and Collinge et al, there should be drastic measures to safeguard the medical and surgical arena from sporadic CJDs and all human TSEs. I only ponder how many sporadic CJDs in the USA are type 2 PrPSc?

http://www.neurology.org/cgi/eletters/60/2/176#535



Diagnosis and Reporting of Creutzfeldt-Jakob Disease


Singeltary, Sr et al. JAMA.2001; 285: 733-734. Vol. 285 No. 6,

February 14, 2001 JAMA

Diagnosis and Reporting of Creutzfeldt-Jakob Disease

To the Editor:

In their Research Letter, Dr Gibbons and colleagues1 reported that the annual US death rate due to Creutzfeldt-Jakob disease (CJD) has been stable since 1985. These estimates, however, are based only on reported cases, and do not include misdiagnosed or preclinical cases. It seems to me that misdiagnosis alone would drastically change these figures. An unknown number of persons with a diagnosis of Alzheimer disease in fact may have CJD, although only a small number of these patients receive the postmortem examination necessary to make this diagnosis. Furthermore, only a few states have made CJD reportable. Human and animal transmissible spongiform encephalopathies should be reportable nationwide and internationally.Terry S. Singeltary, Sr Bacliff, Tex1. Gibbons RV, Holman RC, Belay ED, Schonberger LB. Creutzfeldt-Jakob disease in the United States: 1979-1998. JAMA. 2000;284:2322-2323.

FREE FULL TEXT


http://jama.ama-assn.org/cgi/content/extract/285/6/733?maxtoshow=&HITS=10&hits=10&RESULTFORMAT=&fulltext=singeltary&searchid=1&FIRSTINDEX=0&resourcetype=HWCIT


http://jama.ama-assn.org/cgi/content/full/285/6/733?maxtoshow=&HITS=10&hits=10&RESULTFORMAT=&fulltext=singeltary&searchid=1&FIRSTINDEX=0&resourcetype=HWCIT


2 January 2000

British Medical Journal U.S. Scientist should be concerned with a CJD epidemic in the U.S., as well

http://www.bmj.com/cgi/eletters/320/7226/8/b#6117


15 November 1999

British Medical Journal

vCJD in the USA * BSE in U.S.

http://www.bmj.com/cgi/eletters/319/7220/1312/b#5406


Creutzfeldt Jakob Disease

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


USA PRION UNIT BLOG

http://prionunitusaupdate2008.blogspot.com/


Sunday, April 20, 2008 Progress Report from the National Prion Disease Pathology Surveillance Center April 3, 2008Atypical forms of BSE have emerged which, although rare, appear to be more virulent than the classical BSE that causes vCJD.

see full text ;


http://prionunitusaupdate2008.blogspot.com/2008/04/progress-report-from-national-prion.html


CJD TEXAS (cjd clusters)

http://cjdtexas.blogspot.com/


USA WRITTEN CJD QUESTIONNAIRE ???

http://cjdquestionnaire.blogspot.com/


The statistical incidence of CJD cases in the United States has been revised to reflect that there is one case per 9000 in adults age 55 and older. Eighty-five percent of the cases are sporadic, meaning there is no known cause at present.


http://www.cjdfoundation.org/fact.html


Attending Dr.: Date / Time Admitted : 12/14/97 1228UTMB University of Texas Medical Branch Galveston, Texas 77555-0543 (409) 772-1238 Fax (409) 772-5683 Pathology ReportFINAL AUTOPSY DIAGNOSIS Autopsy' Office (409)772-2858FINAL AUTOPSY DIAGNOSISI. Brain: Creutzfeldt-Jakob disease, Heidenhain variant.


http://creutzfeldt-jakob-disease.blogspot.com/2008/07/heidenhain-variant-creutzfeldt-jakob.html



Friday, December 12, 2008

Creutzfeldt-Jakob disease (CJD) update report Emerging Infections/CJD Published on: 12 December 2008


http://creutzfeldt-jakob-disease.blogspot.com/2008/12/creutzfeldt-jakob-disease-cjd-update.html


Reports and Research

Interference at the EPA

Science and Politics at the U.S. Environmental Protection Agency

The results of these investigations show an agency under siege from political pressures. On numerous issues—ranging from mercury pollution to groundwater contamination to climate change—political appointees have edited scientific documents, manipulated scientific assessments, and generally sought to undermine the science behind dozens of EPA regulations.

http://www.ucsusa.org/scientific_integrity/interference/a-to-z-alphabetical.html


Executive Summary

snip...

The Bush administration’s direct abuse of science—combined with systemic changes to the regulatory system that threaten the integrity of EPA science—highlight the need for strong action by the next president and Con- gress to restore scientific integrity to the agency’s decision making. Only then can the EPA fully mobilize to serve the public good and ensure the nation’s health.

Report: Federal Science and the Public Good

http://www.ucsusa.org/scientific_integrity/interference/interference-at-the-epa.html


posted February 19, 2004 (March 8, 2004 issue) The Junk Science of George W. Bush

The Bush Administration has so violated and corrupted the institutional culture of government agencies charged with scientific research that it could take a generation for them to recover their integrity even if Bush is defeated this fall. Says Princeton University scientist Michael Oppenheimer, "If you believe in a rational universe, in enlightenment, in knowledge and in a search for the truth, this White House is an absolute disaster." .....

February 18, 2004

Preeminent Scientists Protest Bush Administration's Misuse of Science Nobel Laureates, National Medal of Science Recipients, and Other Leading Researchers Call for End to Scientific Abuses .....

snip...

* National Medal of Science † Nobel laureate

http://www.ucsusa.org/news/press_release/preeminent-scientists-protest-bush-administrations-misuse-of-science.html


full text ;


http://sciencebushwhacked.blogspot.com/


http://sciencebushwhacked.blogspot.com/2008/05/junk-science-of-george-w-bush.html



Friday, June 13, 2008 Federal Oversight of Food Safety: FDA Has Provided Few Details GAO-08-909T June 12, 2008 June 12, 2008

FEDERAL OVERSIGHT OF FOOD SAFETY

FDA Has Provided Few Details on the Resources and Strategies Needed to Implement its Food Protection Plan

What GAO Found



snip...


Highlights of GAO-08-909T, a testimony before the Subcommittee on Oversight and Investigations, Committee on Energy and Commerce, House of Representatives

Why GAO Did This Study

The Food and Drug Administration (FDA) is responsible for ensuring the safety of roughly 80 percent of the U.S. food supply, including $417 billion worth of domestic food and $49 billion in imported food annually. Changing demographics and consumption patterns along with an increase in imports have presented challenges to FDA. At the same time, recent outbreaks, such as E. coli from spinach and Salmonella from tomatoes, have undermined consumer confidence in the safety of the food supply. In November 2007, FDA released its Food Protection Plan, which articulates a framework for improving food safety oversight. In January 2008, GAO expressed concerns about FDA’s capacity to implement the Food Protection Plan and noted that more specific information about the strategies and resources needed to implement the plan would facilitate congressional oversight. This testimony focuses on (1) FDA’s progress in implementing the Food Protection Plan, (2) FDA’s proposal to focus inspections based on risk, and (3) FDA’s implementation of previously issued GAO recommendations intended to improve food safety oversight. To address these issues, GAO reviewed FDA documents, such as FDA’s operations plan, and FDA data related to the plan. GAO also interviewed FDA officials regarding the progress made. GAO also analyzed FDA data on domestic and foreign food firm inspections. GAO also analyzed the status of past recommendations.

To view the full product, including the scope and methodology, click on GAO-08-909T. For more information, contact Lisa Shames at (202) 512-3841 or

shamesl@gao.gov.


http://www.gao.gov/highlights/d08909thigh.pdf


SEE FULL TEXT ;

http://www.gao.gov/cgi-bin/getrpt?GAO-08-909T



September 28, 2007

FDA OIG - Food and Drug Administration’s Oversight of Clinical Trials Press release,OIG Releases Report of FDA’s Oversight of Clinical Trials, Concludes Improvement of Information Systems and Processes is Needed, September 2007: "Weaknesses in the Food and Drug Administration’s (FDA) information systems and management processes hinder the agency’s ability to oversee clinical trial inspections. So concludes Inspector General Daniel R. Levinson of the Office of Inspector General (OIG) for the Department of Health and Human Services (HHS) in a report released today...To protect human subjects, federal law requires that all new drugs and medical devices undergo clinical trials to demonstrate their safety and efficacy prior to receiving FDA approval. FDA inspects clinical trials to determine whether sponsors, clinical investigators, and institutional review boards responsible for conducting or overseeing clinical trials for investigational products are complying with relevant regulations. FDA oversees clinical trials through a variety of mechanisms that include protocol reviews and onsite inspections through its Bioresearch Monitoring Program (BiMo). The OIG report focused exclusively on BiMo inspections, an important mechanism for protecting human subjects once a clinical trial is underway. OIG concluded that the FDA does not have a mechanism to identify all clinical trials and Institutional Review Boards (IRBs), which approve, monitor, and review research involving human subjects. Moreover, it lacks a comprehensive database for tracking its inspections of clinical trials. Previous OIG reports found similar weaknesses."

Department of Health and Human Services, Office of Inspector General - The Food and Drug Administration’s Oversight of Clinical Trials, released September 28, 2007. (41 pages, PDF)

http://oig.hhs.gov/publications/docs/press/2007/FDAClinicalTrials3.pdf


>>> Importantly, GAO has noted that public reporting is the means through which the federal government communicates the results of its work to the Congress and the American people.<<< href="http://www.usda.gov/oig/webdocs/sarc070619.pdf">http://www.usda.gov/oig/webdocs/sarc070619.pdf


-MORE


Office of the United States Attorney District of Arizona FOR IMMEDIATE RELEASE For Information Contact Public Affairs February 16, 2007 WYN HORNBUCKLE Telephone: (602) 514-7625 Cell: (602) 525-2681

CORPORATION AND ITS PRESIDENT PLEAD GUILTY TO DEFRAUDING GOVERNMENT’S MAD COW DISEASE SURVEILLANCE PROGRAM

PHOENIX -- Farm Fresh Meats, Inc. and Roland Emerson Farabee, 55, of Maricopa, Arizona, pleaded guilty to stealing $390,000 in government funds, mail fraud and wire fraud, in federal district court in Phoenix. U.S. Attorney Daniel Knauss stated, “The integrity of the system that tests for mad cow disease relies upon the honest cooperation of enterprises like Farm Fresh Meats. Without that honest cooperation, consumers both in the U.S. and internationally are at risk. We want to thank the USDA’s Office of Inspector General for their continuing efforts to safeguard the public health and enforce the law.” Farm Fresh Meats and Farabee were charged by Information with theft of government funds, mail fraud and wire fraud. According to the Information, on June 7, 2004, Farabee, on behalf of Farm Fresh Meats, signed a contract with the U.S. Department of Agriculture (the “USDA Agreement”) to collect obex samples from cattle at high risk of mad cow disease (the “Targeted Cattle Population”). The Targeted Cattle Population consisted of the following cattle: cattle over thirty months of age; nonambulatory cattle; cattle exhibiting signs of central nervous system disorders; cattle exhibiting signs of mad cow disease; and dead cattle. Pursuant to the USDA Agreement, the USDA agreed to pay Farm Fresh Meats $150 per obex sample for collecting obex samples from cattle within the Targeted Cattle Population, and submitting the obex samples to a USDA laboratory for mad cow disease testing. Farm Fresh Meats further agreed to maintain in cold storage the sampled cattle carcasses and heads until the test results were received by Farm Fresh Meats.

Evidence uncovered during the government’s investigation established that Farm Fresh Meats and Farabee submitted samples from cattle outside the Targeted Cattle Population. Specifically, Farm Fresh Meats and Farabee submitted, or caused to be submitted, obex samples from healthy, USDA inspected cattle, in order to steal government moneys.

Evidence collected also demonstrated that Farm Fresh Meats and Farabee failed to maintain cattle carcasses and heads pending test results and falsified corporate books and records to conceal their malfeasance. Such actions, to the extent an obex sample tested positive (fortunately, none did), could have jeopardized the USDA’s ability to identify the diseased animal and pinpoint its place of origin. On Wednesday, February 14, 2007, Farm Fresh Meats and Farabee pleaded guilty to stealing government funds and using the mails and wires to effect the scheme. According to their guilty pleas:

(a) Farm Fresh Meats collected, and Farabee directed others to collect, obex samples from cattle outside the Targeted Cattle Population, which were not subject to payment by the USDA;

(b) Farm Fresh Meats 2 and Farabee caused to be submitted payment requests to the USDA knowing that the requests were based on obex samples that were not subject to payment under the USDA Agreement;

(c) Farm Fresh Meats completed and submitted, and Farabee directed others to complete and submit, BSE Surveillance Data Collection Forms to the USDA’s testing laboratory that were false and misleading;

(d) Farm Fresh Meats completed and submitted, and Farabee directed others to complete and submit, BSE Surveillance Submission Forms filed with the USDA that were false and misleading;

(e) Farm Fresh Meats falsified, and Farabee directed others to falsify, internal Farm Fresh Meats documents to conceal the fact that Farm Fresh Meats was seeking and obtaining payment from the USDA for obex samples obtained from cattle outside the Targeted Cattle Population; and

(f) Farm Fresh Meats failed to comply with, and Farabee directed others to fail to comply with, the USDA Agreement by discarding cattle carcasses and heads prior to receiving BSE test results. A conviction for theft of government funds carries a maximum penalty of 10 years imprisonment. Mail fraud and wire fraud convictions carry a maximum penalty of 20 years imprisonment. Convictions for the above referenced violations also carry a maximum fine of $250,000 for individuals and $500,000 for organizations. In determining an actual sentence, Judge Earl H. Carroll will consult the U.S. Sentencing Guidelines, which provide appropriate sentencing ranges. The judge, however, is not bound by those guidelines in determining a sentence.

Sentencing is set before Judge Earl H. Carroll on May 14, 2007. The investigation in this case was conducted by Assistant Special Agent in Charge Alejandro Quintero, United States Department of Agriculture, Office of Inspector General. The prosecution is being handled by Robert Long, Assistant U.S. Attorney, District of Arizona, Phoenix. CASE NUMBER: CR-07-00160-PHX-EHC RELEASE NUMBER: 2007-051(Farabee) # # #


http://www.usdoj.gov/usao/az/press_releases/2007/2007-051(Farabee).pdf



Thu Dec 6, 2007 11:38

FDA IN CRISIS MODE, AMERICAN LIVES AT RISK


http://www.cidrap.umn.edu/cidrap/content/fs/food-disease/news/dec0407fda.html



FDA SCIENCE AND MISSION AT RISK


http://www.fda.gov/ohrms/dockets/ac/07/briefing/2007-4329b_02_01_FDA%20Report%20on%20Science%20and%20Technology.pdf



In 2007, in one weekly enforcement report, the fda recalled 10,000,000+ pounds of BANNED MAD COW FEED, 'in commerce', and i can tell you that most of it was fed out ;

10,000,000+ LBS. of PROHIBITED BANNED MAD COW FEED I.E. MBM IN COMMERCE USA 2007

Date: March 21, 2007 at 2:27 pm PST 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

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/bbs/topics/enforce/2007/ENF00996.html



Subject: MAD COW FEED RECALL USA SEPT 6, 2006 1961.72 TONS IN COMMERCE AL, TN, AND WV Date: September 6, 2006 at 7:58 am PST

snip... see listings and references of enormous amounts of banned mad cow protein 'in commerce' in 2006 and 2005 ;

see full text ;

Friday, April 25, 2008

Substances Prohibited From Use in Animal Food or Feed [Docket No. 2002N-0273] (Formerly Docket No. 02N-0273) RIN 0910-AF46



http://madcowfeed.blogspot.com/2008/04/substances-prohibited-from-use-in.html



SPECIFIED RISK MATERIALS



http://madcowspontaneousnot.blogspot.com/2008/02/specified-risk-materials-srm.html



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


http://cjdmadcowbaseoct2007.blogspot.com/2008/04/srm-mad-cow-recall-406-thousand-pounds.html



Greetings,


AFTER 8 years of the Bush Administration covering up mad cow disease of all strains here in the USA, i only ponder what the newly elected incoming President will do ???

WILL it be mad cow big ag political junk science as usual $$$

OR WILL someone finally take control from BIG AG ???

I would kindly suggest gutting the USDA, FSIS, APHIS, FDA et al to the core.

BRING back sound science !



Terry S. Singeltary Sr.

P.O. Box 42

Bacliff, Texas USA 77518

Labels: , , , , , , ,

Friday, December 12, 2008

The prion strain phenomenon: Molecular basis and unprecedented features

Biochim Biophys Acta. Author manuscript; available in PMC 2008 December 9. Published in final edited form as: Biochim Biophys Acta. 2007 June; 1772(6): 681–691. Published online 2006 December 15. doi: 10.1016/j.bbadis.2006.12.006. PMCID: PMC2597801 NIHMSID: NIHMS25810

Copyright notice and Disclaimer


The prion strain phenomenon: Molecular basis and unprecedented features


Rodrigo Morales,1,2 Karim Abid,1 and Claudio Soto1# 1 Protein Misfolding Disorders Laboratory, George and Cynthia Mitchell Center for Neurodegenerative diseases, Departments of Neurology, Neuroscience & Cell Biology and Biochemistry & Molecular Biology, University of Texas Medical Branch, 301 University Boulevard, Galveston, Texas, 77555-0646, USA 2 Facultad de Ciencias, Universidad de Chile, Santiago, Chile #To whom correspondence should be addressed at Email: clsoto@utmb.edu The publisher's final edited version of this article is available at Biochim Biophys Acta. See other articles in PMC that cite the published article.


Abstract


Prions are unconventional infectious agents responsible for transmissible spongiform encephalopathies. Compelling evidences indicate that prions are composed exclusively by a misfolded form of the prion protein (PrPSc) that replicates in the absence of nucleic acids. One of the most challenging problems for the prion hypothesis is the existence of different strains of the infectious agent. Prion strains have been characterized in most of the species. Biochemical characteristics of PrPSc used to identify each strain include glycosylation profile, electrophoretic mobility, protease resistance, and sedimentation. In vivo, prion strains can be differentiated by the clinical signs, incubation period after inoculation and the vacuolation lesion profiles in the brain of affected animals. Sources of prion strain diversity are the inherent conformational flexibility of the prion protein, the presence of PrP polymorphisms and inter-species transmissibility. The existence of the strain phenomenon is not only a scientific challenge, but it also represents a serious risk for public health. The dynamic nature and inter-relations between strains and the potential for the generation of a very large number of new prion strains is the perfect recipe for the emergence of extremely dangerous new infectious agents.


snip...


BSE has not only been transmitted to humans. The extensive use of cow-derived material for feeding other animals led to the generation of new diseases in exotic felines such as tiger and cheetah, non human primates, and domestic cats [52,57–60]. As it was mentioned before, the transmission of BSE into these different species could create many new prion strains, each one of them with particular biological and biochemical characteristics and thus a potentially new hazard for human health. Successful transmission of BSE in pigs has been described [61,62] and also in transgenic mice expressing pig PrP (PoPrP) [63]. Porcine derivates are widely consumed and the hypothetic case of “mad pigs” could increase the events of zoonotic transmission of prions to humans. Fortunately, transmission of BSE to pigs is possible only in very drastic conditions, not likely to be occurring naturally [62,63]. More frightening is perhaps the possibility that BSE has been passed into sheep and goats. Studies have already shown that this transmission is possible and actually relatively easy and worrisomely produces a disease clinically similar to scrapie [64]. The cattle origin of this new scrapie makes possible that the new strain may be transmissible to humans. Transmission experiments of BSE infected sheep brain homogenate into human transgenic animal models are currently ongoing in several laboratories. It is very important to note that all materials generated by transmission of BSE in experimental and natural cases show similar biochemical behavior compared to the original inoculum [65], suggesting that all these new generated infectious agents could potentially be hazardous for humans. The origin of BSE is still a mystery. Abundant evidence supports the hypothesis that BSE was produced by cattle feeding with scrapie derivated material [66,67], indicating that bovine PrPSc might be a “conformational intermediary” between ovine PrPSc and human PrPC.


There is currently no mean to predict which will be the conformation of a newly generated strain and how this new PrPSc conformation could affect other species. One interesting new prion disease is CWD, a disease affecting farm and wild species of cervids [68,69]. The origin of CWD and its potential to transmit to humans are currently unknown. This is worrisome, considering that CWD has became endemic in some parts of USA and the number of cases continues to increase [69]. It is presumed that a large number of hunters in the US have been in contact or consumed CWD-infected meat [70]. CWD transmissibility studies have been performed in many species in order to predict how this disease could be spread by consumption of CWD meat [71–73]. In these studies, a special attention has been done to scavenging animals [74], which are presumed to be exposed to high concentration of cervid prions, resulting in the putative generation of many new forms of TSEs. Fortunately negative results were obtained in one experiment done in raccoons infected with CWD [74]. Transmission of CWD to humans cannot be ruled out at present and a similar infective episode to BSE involving CWD could result in catastrophic events, spreading the disease in a very dangerous way through the human population. No clinical evidence linking CWD exposed humans and CJD patients have been found [70], but experimental inoculation of CWD prions into squirrel monkeys propagated the disease [71]. It is important to mention that the species barrier between humans and cervids appears to be greater than with cattle, as judged by experiments with transgenic mice models [75]. Finally, it is important to be aware about CWD transmissibility to other species in which a “conformational intermediary” could be formed, facilitating human infection.


SNIP...


VI. Unique features of prion strains

The biological and infectious characteristics of prions are dramatically different to the conventional infectious agents. These differences are manifested in the prion strains phenomenon in unique and unprecedented features, such as for example strain adaptation and memory, the coexistence and competition of prion strains, among others. In this section, some of these interesting phenomena will be briefly described.

Adaptation of Prion strains

Interspecies transmission of prions could result in the emergence of more than one variety of infectious material. All new collected infectious agents could present particular strain characteristics. That is the case of DY and HY prion strains generation [13,16]. When interspecies transmission of prions occurs, serial passages in the new host are needed in order to stabilize the characteristics of new generated infectious material. In the case of TME transmission in hamsters, at least four serial passages in the new species were required for stabilization [13]. The first passage was characterized by long incubation periods and a dominance of a 19 KDa fragment when newly obtained PrPSc was analyzed after PK digestion. In the three first passages, clinical symptoms were not characteristic of the hamster-adapted HY or DY TME strains. This phenotype was attributed to the combination effects of both strains replicating simultaneously. Thereafter, each of the strains was stabilized in some of the animals and once they are adapted and stabilized, they can be serially propagated in vivo and the characteristics are maintained. It is accepted that both strains present differential conversion kinetics in vitro, with DY being the slowest and HY the fastest [124]. For this reason, in order to select efficiently this prion strain, limit dilutions must be performed [13]. In that way, the most abundant and less convertible DY is favored against the less abundant but fastest HY strain.


Co-existence of prion strains


Related to the above, it has been shown that two or more prion strains can co-exist in natural cases of TSE. Co-existence of prion strains has been found in sporadic cases of CJD [113, 125]. Analyses of several sCJD tissue showed that different biochemical profiles of PrPSc could be found in different brain areas from the same patient [113]. Co-existence of prion strains was mainly observed in patient heterozygous for codon 129 [113]. As many as 50% of these patients present different types of PrPSc in their brains, whereas 9% of MM patients were positive for co-existence of strains. On the other hand, more than one PrPSc type was not observed in VV patients [113].


The biochemical and structural properties of the protein seem to be the major cause of this differential distribution. This observation may explain why sCJD is so heterogeneous in terms of clinical manifestation [34,126,127]. In a recent publication by Bishop et al. [107], vCJD infected transgenic mice expressing human PrPC, present changes in their PrPSc and vacuolation patterns in the brain according to their polymorphic classification for codon 129.


Competition of prion strains


In particular experimental conditions, some prion strains can extend their specific incubation period when co-infected with another strain. Long incubation period prions increase the incubation period of “faster” prions. This phenomenon of “competition of prion strains” has been observed in mice and hamster. In mice, competition between 22A and 22C strains was reported in 1975 by Dickinson et al. [128]. In this study, RIII mice (homozygous for sincs7 allele) were used. 22A and 22C showed long and short incubation period (550 and 230 days), respectively. When 22C strain was intraperitoneally inoculated 100, 200 and 300 days after intraperitoneal administration of the 22A agent, all three experimental groups resulted in Morales et al. Page 8 Biochim Biophys Acta. Author manuscript; available in PMC 2008 December 9. NIH-PA Author Manuscript NIH-PA Author Manuscript NIH-PA Author Manuscript incubation periods and lesion patterns matching 22A prions, suggesting that 22C prions were degraded or excreted, in animals previously infected by 22A. Similar results were obtained by Kimberlin and Walker in 1985 [129] using a different strain of sincs7 mice. These authors treated mice using 22A and 22C prion strain. Before inoculation, 22A was treated with different chemical and physical agents in order to see if the “competitor” or “blocking” characteristics of 22A were maintained. From all treatments, 12M urea was shown to almost abolish the blocking properties of 22A agent. This information suggests that infective properties of long incubation period agent are strictly necessary in order to increase the incubation period of faster prions.


In hamster, similar observations were reported using DY and HY [130]. DY prion strain was inoculated 30 and 60 days prior intraperitoneal inoculation of HY at three different doses. When incubation periods of HY inoculated control group were compared with the animals inoculated at 60 days with DY, significant differences in the incubation periods were found, especially when HY prions were administrated in a higher dose [130]. On the other hand no differences were observed in the case of intranerve inoculation, revealing that competition phenomenon occurs only when peripheral inoculation is performed. These results are surprising considering the fact that DY was reported not to be infectious when intraperitoneally inoculated in hamsters [130]. This data suggest that replication of DY is occurring in peripheral tissues but is not able to reach the central nervous system.


In general, the principal variables that need to be observed for a successful competition are the route of infection, the interval between injections and the particular strains and doses of agent used. Prolongation of incubation periods in TSE are therapeutically beneficial and several strategies are under development to reach this aim, including antibodies, beta-sheet breakers, and other chemical agents [131–133]. The experimental evidence described above suggests that prions could be potentially useful for this purpose. In order to prevent spread of prion disease in cattle or humans, prion strains with incubation periods longer than species’ lifespan could be used to slowdown the replication of BSE or vCJD prions.


VII. Concluding Remarks


The existence of different strains of an infectious agent composed exclusively of a protein has been one of the most puzzling issues in the prion field. If is already difficult to understand how a protein can adopt two stable and different folded structures and that one of them can transform the other one into itself, it is unthinkable that the misfolded form can in turn adopt multiple conformations with distinct properties. Yet, compelling scientific evidence support the idea that PrP can adopt numerous folding patterns that can faithfully replicate and produce different diseases. The existence of the strain phenomenon is not only a scientific challenge, but it also represents a serious risk for public health. The dynamic nature and inter-relations between strains and the potential for the generation of many new prion strains depending on the polymorphisms and the crossing of species barrier is the perfect recipe for the emergence of extremely dangerous new infectious agents. Although, substantial progress has been made in understanding the prion strains phenomenon, there are many open questions that need urgent answers, including: what are the structural basis of prion strains?; how are the phenomena of strain adaptation and memory enciphered in the conformation of the prion agent?; to what species can a given prion strain be transmissible?; what other cellular factors control the origin and properties of prion strains?. ...SNIP...END


http://www.pubmedcentral.nih.gov/articlerender.fcgi?tool=pmcentrez&artid=2597801&rendertype=abstract



http://www.pubmedcentral.nih.gov/picrender.fcgi?artid=2597801&blobtype=pdf



Sunday, March 16, 2008

MAD COW DISEASE terminology UK c-BSE (typical), atypical BSE H or L, and or Italian L-BASE


http://bse-atypical.blogspot.com/2008/03/mad-cow-disease-terminology-uk-c-bse.html



HUMAN and ANIMAL TSE Classifications i.e. mad cow disease and the UKBSEnvCJD only theory JUNE 2008

snip...

Tissue infectivity and strain typing of the many variants Manuscript of the human and animal TSEs are paramount in all variants of all TSE. There must be a proper classification that will differentiate between all these human TSE in order to do this. With the CDI and other more sensitive testing coming about, I only hope that my proposal will some day be taken seriously. ...

snip...

http://cjdmadcowbaseoct2007.blogspot.com/2008/06/human-and-animal-tse-classifications-ie.html



Friday, December 05, 2008

Detection of Prion Infectivity in Fat Tissues of Scrapie-Infected Mice

http://scrapie-usa.blogspot.com/2008/12/detection-of-prion-infectivity-in-fat.html



Evaluation of the Human Transmission Risk of an Atypical Bovine Spongiform Encephalopathy Prion Strain

Qingzhong Kong,1* Mengjie Zheng,1 Cristina Casalone,2 Liuting Qing,1 Shenghai Huang,1? Bikram Chakraborty,1 Ping Wang,1 Fusong Chen,1 Ignazio Cali,1 Cristiano Corona,2 Francesca Martucci,2 Barbara Iulini,2 Pierluigi Acutis,2 Lan Wang,1 Jingjing Liang,1 Meiling Wang,1 Xinyi Li,1 Salvatore Monaco,3 Gianluigi Zanusso,3 Wen-Quan Zou,1 Maria Caramelli,2 and Pierluigi Gambetti1*
Department of Pathology, Case Western Reserve University, Cleveland, Ohio 44106,1 CEA, Istituto Zooprofilattico Sperimentale, 10154 Torino, Italy,2 Department of Neurological and Visual Sciences, University of Verona, 37134 Verona, Italy3
*Corresponding author. Mailing address: Department of Pathology, Case Western Reserve University, Cleveland, OH 44106. Phone for Pierluigi Gambetti: (216) 368-0586. Fax: (216) 368-2546. E-mail: pxg13@case.edu . Phone for Qingzhong Kong: (216) 368-1756. Fax: (216) 368-2546. E-mail: qxk2@case.edu
?Present address: Department of Patient Education and Health Information, Cleveland Clinic Foundation, Cleveland, OH 44195.
Received November 30, 2007; Accepted January 16, 2008.

Bovine spongiform encephalopathy (BSE), the prion disease in cattle, was widely believed to be caused by only one strain, BSE-C. BSE-C causes the fatal prion disease named new variant Creutzfeldt-Jacob disease in humans. Two atypical BSE strains, bovine amyloidotic spongiform encephalopathy (BASE, also named BSE-L) and BSE-H, have been discovered in several countries since 2004; their transmissibility and phenotypes in humans are unknown. We investigated the infectivity and human phenotype of BASE strains by inoculating transgenic (Tg) mice expressing the human prion protein with brain homogenates from two BASE strain-infected cattle. Sixty percent of the inoculated Tg mice became infected after 20 to 22 months of incubation, a transmission rate higher than those reported for BSE-C. A quarter of BASE strain-infected Tg mice, but none of the Tg mice infected with prions causing a sporadic human prion disease, showed the presence of pathogenic prion protein isoforms in the spleen, indicating that the BASE prion is intrinsically lymphotropic. The pathological prion protein isoforms in BASE strain-infected humanized Tg mouse brains are different from those from the original cattle BASE or sporadic human prion disease. Minimal brain spongiosis and long incubation times are observed for the BASE strain-infected Tg mice. These results suggest that in humans, the BASE strain is a more virulent BSE strain and likely lymphotropic.

http://www.pubmedcentral.nih.gov/articlerender.fcgi?artid=2268471



Thursday, December 04, 2008 2:37 PM

"we have found that H-BSE can infect humans."

personal communication with Professor Kong. ...TSS



November 25, 2008

Update On Feed Enforcement Activities To Limit The Spread Of BSE

http://madcowfeed.blogspot.com/2008/11/november-2008-update-on-feed.html



"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."

just another one of those sporadic CJD coincidences i suppose $$$

NOT to forget ;

Thursday, June 05, 2008

Review on the epidemiology and dynamics of BSE epidemics

Vet. Res. (2008) 39:15 www.vetres.org DOI: 10.1051/vetres:2007053 c INRA, EDP Sciences, 2008 Review article

snip...

And last but not least, similarities of PrPres between Htype BSE and human prion diseases like CJD or GSS have been put forward [10], as well as between L-type BSE and CJD [17]. These findings raise questions about the origin and inter species transmission of these prion diseases that were discovered through the BSE active surveillance.

snip...

Cases of atypical BSE have only been found in countries having implemented large active surveillance programs. As of 1st September 2007, 36 cases (16 H, 20 L) have been described all over the world in cattle: Belgium (1 L) [23], Canada (1 H)15, Denmark (1 L)16, France (8 H, 6 L)17, Germany (1 H, 1 L) [13], Italy (3 L)18, Japan (1 L) [71], Netherlands (1 H, 2 L)19, Poland (1 H, 6 L)20, Sweden (1 H)21, United Kingdom (1 H)22, and USA (2 H)23. Another H-type case has been found in a 19 year old miniature zebu in a zoological park in Switzerland [56]. It is noteworthy that atypical cases have been found in countries that did not experience classical BSE so far, like Sweden, or in which only few cases of classical BSE have been found, like Canada or the USA.

And last but not least, similarities of PrPres between Htype BSE and human prion diseases like CJD or GSS have been put forward [10], as well as between L-type BSE and CJD [17]. These findings raise questions about the origin and inter species transmission of these prion diseases that were discovered through the BSE active surveillance.

full text 18 pages ;

http://www.vetres.org/index.php?option=article&access=standard&Itemid=129&url=/articles/vetres/pdf/2008/04/v07232.pdf


please see full text ;

http://bse-atypical.blogspot.com/2008/06/review-on-epidemiology-and-dynamics-of.html


***Atypical forms of BSE have emerged which, although rare, appear to be more virulent than the classical BSE that causes vCJD.***

Progress Report from the National Prion Disease Pathology Surveillance Center

An Update from Stephen M. Sergay, MB, BCh & Pierluigi Gambetti, MD

April 3, 2008

http://www.aan.com/news/?event=read&article_id=4397&page=72.45.45



Friday, December 12, 2008

Prions in Milk from Ewes Incubating Natural Scrapie

http://scrapie-usa.blogspot.com/2008/12/prions-in-milk-from-ewes-incubating.html



Attending Dr.: Date / Time Admitted : 12/14/97 1228

UTMB University of Texas Medical Branch Galveston, Texas 77555-0543 (409) 772-1238 Fax (409) 772-5683 Pathology Report

FINAL AUTOPSY DIAGNOSIS Autopsy' Office (409)772-2858

FINAL AUTOPSY DIAGNOSIS

I. Brain: Creutzfeldt-Jakob disease, Heidenhain variant.

http://creutzfeldt-jakob-disease.blogspot.com/2008/07/heidenhain-variant-creutzfeldt-jakob.html



0C3.01

Transmission of atypical BSE to Microcebus murinus, a non-human primate: Development of clinical symptoms and tissue distribution of PrPres

Background: Atypical BSE cases have been observed in Europe, Japan and North America. They differ in their PrPres profiles from those found in classical BSE. These atypical cases fall into 2 types, depending on the molecular mass of the unglycosylated PrPres band observed by Western blot: the L -type (lower molecular mass than the typical BSE cases) and H-type (higher molecular mass than the typical BSE cases).

Objectives and Methods: In order to see if the atypical BSE cases were transmissible to primates, either animals (were intracerebrally inoculated with 50 ul of a 10% brain homogenates of two atypical French BSE case, a H-type (2 males and 2 females) and a L-type (2 males and 2 females).

Results: Only one of the four lemurs challenged with H-type BSE died without clinical signs after 19 months post inoculation (mpi), whereas all the 4 animals inoculated with L -type BSE died at 19 mpi (2 males) and 22 mpi (2 females). Three months before their sacrifice, they developed blindness, tremor, abnormal posture, incoordinated movements, balance loss. Symptoms got worse according to the disease progression, until severe ataxia. The brain tissue were biochemically and immunocytochemically investigated for PrPres. For the H-type, spongiform changes without PrPres accumulation were observed in the brainstem. However Western blot analysis did not allow to detect PrPres into the brain. For the L-type, severe spongiosis was evidenced into the thalamus, the striatum, the mesencephalon, and the brainstem. whereas into the cortex the spongiosis was evidenced, but the Vacuolisation was weaker. Strong deposits of PrPres were detected by western blot, PET-blot and immunocytochemistry in the CNS: dense accumulation was observed into the thalamus, the striatum, and the hippocampus whereas in the cerebral cortex, PrPres was prominently accumulated in plaques. Western blot analysis also readily confirmed the presence of protease-resistant prion protein.

Conclusions: L-type infected lemurs showed survival times considerably shorter than for classical BSE strain, indicating that the disease is caused by a very virulent distinct prion strain in a model of non human primate.

http://www.neuroprion.org/resources/pdf_docs/conferences/prion2008/abstract-book-prion2008.pdf



P7.09

Biochemical screening for identification of atypical bse in belgium, 1999-present

Authors

Alexandre DobIy: Caroline Rodeghiero, Riet Geeroms; Stephanie Durand, Jessica De Sloovere, Emanuel Yanopdenbosch, Stefan Roels,

Content

Background: Recently atypical forms of BSE have been described. Western blot analyses showed that, in comparison to the classic BSE (C-type), they are demonstrable by a higher or lower molecular weight of the unglycosylated PrPres. They Viere thus named H-type and L-type BSE (L-type is also called BASE). In addition they show a lower proportion of diglycosylated PrPres than C-type. These emerging types represent different strains of BSE. They show unique incubation periods and histological lesions. Such types have been described on different continents. Indeed they might correspond to "sporadic" forms of BSE. In 2004 we already described one L-type in Belgium.

Objective: We retrospectively analysed the bovines at least 7-year-old in the Belgian archive of BSE ­diagnosed cattle in order to determine the prevalence of the two types of atypical BSE in Belgium.

Methods: We analysed homogenates from 39 bovines of 93 months old in median (min: 84, max: 181 months). The most recent one was diagnosed in 2006. We used Western blot with a panel of anti-PrP antibodies (Ab). They detect different regions of the PrP protein, from N-terminal to C-terminal: 12B2, 9A2, Sha31. SAFB4, 94B4. Their combination is aimed at an efficient typing diagnostic. We detected bound Ab with SuperSignal West Dura (Pierce) and analysed PrPres, signals with an image-analysis software (Quantity One, Bio-Rad).

Results: The results are still under analysis. We will detail the most crucial characteristics for typing PrPres. These include 1) the apparent molecular mass of the an-, mono- and diglycosylated bands, 2) the binding affinity to the five Ab (e.g.12B2 for H-type), 3) the presence of a fourth (unglycosylated) band and 4) the glycoprofile based on the relative proportions of the visible bands.

Discussion: The emergence of atypical types of BSE is partially due to a better knowledge of prion strains and more efficient diagnostic techniques. As the area in the brain where the PrPres is deposited can differ drastically between the types, it is essential to ascertain that the sampling techniques and analyses are adapted to these new types. As these new strains seem more virulent than classic types, they represent one of the next challenges in the field of prions.

http://www.neuroprion.org/resources/pdf_docs/conferences/prion2008/abstract-book-prion2008.pdf



http://www.prion2008.com/



Tuesday, November 11, 2008

Transmission of atypical bovine prions to mice transgenic for human prion protein

DOI: 10.3201/eid1412.080941

http://bse-atypical.blogspot.com/2008/11/transmission-of-atypical-bovine-prions.html


Wednesday, August 20, 2008

Bovine Spongiform Encephalopathy Mad Cow Disease typical and atypical strains, was there a cover-up ?

http://bse-atypical.blogspot.com/2008/08/bovine-spongiform-encephalopathy-mad.html


Tuesday, June 3, 2008 SCRAPIE USA UPDATE JUNE 2008 NOR-98 REPORTED PA

http://nor-98.blogspot.com/2008/06/scrapie-usa-update-june-2008-nor-98.html


SCRAPIE USA

http://scrapie-usa.blogspot.com/


0C7.04

North American Cervids Harbor Two Distinct CWD Strains

Authors

Angers, R. Seward, T, Napier, D., Browning, S., Miller, M., Balachandran A., McKenzie, D., Hoover, E., Telling, G. 'University of Kentucky; Colorado Division of Wildlife, Canadian Food Inspection Agency; University Of Wisconsin; Colorado State University.

Content

Despite the increasing geographic distribution and host range of CWD, little is known about the prion strain(s) responsible for distinct outbreaks of the disease. To address this we inoculated CWD-susceptible Tg(CerPrP)1536+/· mice with 29 individual prion samples from various geographic locations in North America. Upon serial passage, intrastudy incubation periods consistently diverged and clustered into two main groups with means around 210 and 290 days, with corresponding differences in neuropathology. Prion strain designations were utilized to distinguish between the two groups: Type I CWD mice succumbed to disease in the 200 day range and displayed a symmetrical pattern of vacuolation and PrPSc deposition, whereas Type II CWD mice succumbed to disease near 300 days and displayed a strikingly different pattern characterized by large local accumulations of florid plaques distributed asymmetrically. Type II CWD bears a striking resemblance to unstable parental scrapie strains such as 87A which give rise to stable, short incubation period strains such as ME7 under certain passage conditions. In agreement, the only groups of CWD-inoculated mice with unwavering incubation periods were those with Type I CWD. Additionally, following endpoint titration of a CWD sample, Type I CWD could be recovered only at the lowest dilution tested (10-1), whereas Type II CWD was detected in mice inoculated with all dilutions resulting in disease. Although strain properties are believed to be encoded in the tertiary structure of the infectious prion protein, we found no biochemical differences between Type I and Type II CWD. Our data confirm the co·existence of two distinct prion strains in CWD-infected cervids and suggest that Type II CWD is the parent strain of Type I CWD.


see page 29, and see other CWD studies ;


http://www.neuroprion.org/resources/pdf_docs/conferences/prion2008/abstract-book-prion2008.pdf


Sunday, November 23, 2008

PRION October 8th - 10th 2008 Book of Abstracts

http://bse-atypical.blogspot.com/2008/11/prion-october-8th-10th-2008-book-of.html


Sunday, September 07, 2008

CWD LIVE TEST, and the political aspects or fallout of live testing for BSE in cattle in the USA

http://chronic-wasting-disease.blogspot.com/2008/09/cwd-live-test-and-political-aspects-or.html


Saturday, October 18, 2008 WYOMING STAR VALLEY MOOSE TESTS POSITIVE FOR CWD

http://chronic-wasting-disease.blogspot.com/2008/10/wyoming-star-valley-moose-tests.html


http://chronic-wasting-disease.blogspot.com/



TSS

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