CJD...Straight talk with...James Ironside...and...Terry Singeltary... 2009

News abstract

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Nature Medicine 15, 834 - 835 (2009) doi:10.1038/nm0809-834

Straight talk with...James Ironside

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Abstract

Would you entrust your brain to a bank? Well, many people do after they die, and such brain banks—often funded by government agencies or disease charities—are essential for neuroscience research. They collect and store the healthy and diseased brain specimens that neuroscientists need to explore neurological disorders such as Alzheimer's disease, schizophrenia and autism. Each brain bank typically has a limited supply of samples and tends to operate fairly independently. This means that researchers often have to trawl through numerous brain banks to find their desired specimens. Furthermore, there is a general shortage of brain samples. To help resolve these issues in the UK, James Ironside, professor of clinical neuropathology at the University of Edinburgh, was appointed in June as the director of the new UK Brain Banks Network. An expert in human prion diseases, particularly Creutzfeldt-Jakob disease (CJD), Ironside knows all about brain banks. He established the Brain and Tissue Bank at the UK's National CJD Surveillance Unit and is involved in the Sudden Death Brain and Tissue Bank at the University of Edinburgh. Jon Evans recently caught up with Ironside to discuss his new leadership position and how the brain network will benefit neuroscience research.


http://www.nature.com/nm/journal/v15/n8/abs/nm0809-834.html



straight talk with Terry Singeltary ;


Dr. James Ironside ask ;


> Would you entrust your brain to a bank?


WE all did here in the USA, and see what almost happened below ;


NIH may destroy human brain collection

By Steve Mitchell Medical Correspondent

Washington, DC, Mar. 24 (UPI) -- The National Institutes of Health may discard part or all of a rare collection that includes hundreds of human brain samples from patients that suffered from a disorder similar to mad cow disease -- unless another researcher or institution takes them on, United Press International has learned.

Several scientists said the collection, which is held by the NIH's National Institute for Neurological Disorders and Stroke in Bethesda, Md. -- and includes brains and other tissue samples from people afflicted with the brain-wasting illness Creutzfeldt Jakob disease -- is irreplaceable and could even provide insight into treatments for the fatal disorder. Currently, there is no cure for CJD and patients typically die within a year after symptoms begin.

However, NIH officials in control of the collection's fate told UPI the remaining samples are of little scientific value and may be disposed of if researchers outside the agency do not claim it. That position stands in sharp contrast with CJD experts who thought the collection should be preserved.

"It's invaluable," said Dr. Paul Brown, former medical director of the NIH's Laboratory for Central Nervous System Studies, whose expertise is in CJD and mad cow disease (also known as bovine spongiform encephalopathy, or BSE).

The collection is badly in need of organization and no one is certain how many brains or other tissue samples it contains, said Brown, who worked with the collection since its inception in the 1960's until his retirement last year. There could be brains, blood, spinal fluid and various other tissues from 1,000 people or more, he said. Some of the specimens would be of scientific use today, he said.

"This collection has the unique value of stretching back to the beginning of when these diseases were discovered," Brown told UPI, noting that the first samples were obtained in 1963. "It would be as though you had in your hands the possibility of finding out when AIDS started."

Bruce Johnson, a former technician at the CNSS lab who worked extensively with the collection before he retired in 2003, told UPI he was told "in two years they (NIH officials)are going to destroy it, if nobody wants it."

Eugene Major, acting director of the basic neuroscience program at the NIH, said no specific timeframe had been established.

"We have not set a firm deadline date," Major told UPI. "We are working very hard with investigators that we know in order to be able to make sure that whatever we deem is valuable is potentially kept here." Some samples already have been determined not to have any research value and have been "removed and disposed of," he said.

Others samples have been given out to Dr. David Asher at the Food and Drug Administration and Pierluigi Gambetti at the National Prion Disease Pathology Surveillance Center in Cleveland, Ohio.

Major maintained the remaining collection was not particularly valuable for research. "Whatever had been collected here that has not already been distributed to responsible investigators who could use them really has very little remaining value," he said.

Neither Asher nor Gambetti returned phone calls from UPI, but Brown said he thought Asher had received only a dozen or two samples at most and Gambetti had not received much at all.

Neil Cashman, a brain-disease researcher at the University of Toronto's Center for Research in Neurodegenerative Diseases -- who has tried to obtain the collection from the NIH -- said it was priceless.

"It would be like destroying an art museum," Cashman told UPI. "There's all this information and insight that's locked up in these tissues and if it's destroyed it will be lost forever."

The Memorial Institute for Neurodegenerative Diseases Inc., a non-profit organization consisting of more than 40 university and institute researchers from the United States, Canada, United Kingdom and France, also thinks the brain collection is invaluable.

"It is the opinion of the Board of Directors ... of The MIND Inc., that the ... brain bank should not be broken up nor destroyed," said Harry E. Peery, MIND's executive director, in a letter to UPI. "We believe that this collection is of inestimable research value and should be kept intact."

The institute, at the University of Saskatchewan in Saskatoon, applied for possession of the collection in early 2004, but received a letter from the NINDS indicating the fate of the collection had not yet been determined.

"We have heard nothing further since that time" and continue to be interested in acquiring the complete collection, Peery said.

CJD belongs to a group of rare, brain-wasting disorders that are little understood, incurable and fatal. This includes mad cow disease in cows, chronic wasting disease in deer and elk. The most infamous of these illnesses in humans is variant CJD, which people can contract from eating beef products infected with the mad-cow pathogen.

Although vCJD has infected more than 154 people worldwide, only one case has ever been detected in the United States -- in a Florida woman who is thought to have contracted the disease while living in the United Kingdom. However, the NIH brain samples have never been screened for vCJD -- something Johnson thinks is critically important.

"No one has ever looked to see if any American (in the collection) in the past had variant CJD," Johnson said. "You think it would be required that they do that. You think it would be a Congressional mandate that they test these brains: 'Let's see if we've got this disease in our country.'"

Johnson noted at least one brain in the collection he personally had examined -- from a French woman collected in 1971 -- showed evidence of possible vCJD infection, but the sample needed further study to be sure.

Other samples in the collection include the brains of patients who were only 16 years old when they were diagnosed with CJD. This would be unusual for sporadic CJD, because generally it strikes those over age 60. Variant CJD, on the other hand, typically occurs in patients in their 20s or younger.

"I thought it was absolutely vital (to test these brains)," Johnson said. "Maybe there's a dozen cases in there of variant CJD."

Major disagreed. "There's really no reason to do that," he said. "The effort it would take to screen those samples ... would not give us any new insights into variant CJD beyond what it is we already know."

Johnson said he was frustrated with the NIH administration's lack of interest in preserving the collection or testing for vCJD. "They don't understand," he said, "they honest-to-god don't understand what it's all about."

Patient advocates also objected to the possible destruction of the brains.

Terry Singeltary, whose mother died of a type of CJD called Heidenhain variant in 1997, said he is outraged and families of other CJD victims probably will be, too.

"A lot of these families went through a lot of heartache and a lot of trouble to get these brain samples to the NIH," Singeltary told UPI. "Now they're just going to discard them because they're not of scientific use? That's just asinine. That stuff is valuable information."

Graham Steel, vice-chair of the Human BSE Foundation in the United Kingdom, told UPI, "The potential loss of such important tissue samples would be a massive blow for TSE (the group of diseases that includes CJD and BSE) research in the United States. This should not be allowed to happen."

Singeltary noted there currently is no cure for these diseases. "If you don't have any answers yet, why would you throw these specimens away?" he asked.

He added that more sensitive tests are just becoming available and could help determine the origin of some of the CJD cases. "We've all been sitting around waiting for more sensitive tests to get validated because we want answers," he said.

"You know, it must be an embarrassment," Johnson said. "Some Senator is going to eventually say 'What is NIH doing about mad cow disease?' And people are going to scratch their heads and say 'not much'." He added, "What's going to happen (is) one of these senators or their wife is going to develop spontaneous CJD one day and ... there's going to be hell raised and they're going to ask, 'Why isn't NIH working on this?'"

--

http://www.washtimes.com/upi-breaking/20050323-053919-8481r.htm



http://www.accessmylibrary.com/coms2/summary_0286-8492054_ITM



http://www.promedmail.org/pls/otn/f?p=2400:1202:9557296506089968094::NO::F2400_P1202_CHECK_DISPLAY,F2400_P1202_PUB_MAIL_ID:X,28585



SCIENCE NEWS

Groups seek to save NIH brain collection

Published: April 1, 2005 at 4:48 PM By STEVE MITCHELL, Medical Correspondent

WASHINGTON, April 1 (UPI) -- Scientists, consumer groups and patient-advocates have embarked upon efforts -- including petitioning members of Congress and seeking storage space at a Canadian university -- to prevent the National Institutes of Health from destroying an irreplaceable collection of human brains from patients afflicted with a condition similar to mad cow disease.

As United Press International reported last week, the NIH has begun shopping for a new home for its collection of brains, spinal fluid and other tissues from hundreds of patients around the world who died from Creutzfeldt Jakob disease -- an incurable, fatal, brain-wasting illness. The collection dates back to 1963 and the consensus among scientists in this field is it is invaluable for research and could provide insights that might aid in developing diagnostic tests, treatments or cures for CJD.

NIH officials, however, maintain the remaining samples in the collection -- stored in some 30 freezers by the National Institute for Neurological Disorders and Stroke in Bethesda, Md. -- are of little value and may be disposed of if researchers or institutions do not come forward to claim them.

Families of patients who died of CJD have reacted with outrage, concerned that the effort mounted to collect the brains in the first place has been all for naught. Several have contacted their respective members of Congress and urged them to step in.

"The brains and brain tissue were sent to NIH in good faith for future research and destroying them is an outrage," Terry Singeltary, a patient advocate in Bacliff, Texas, wrote in a letter to Sen. Kay Bailey Hutchinson, R-Texas, and several other members of the state's congressional delegation. Singeltary's mother died of a type of CJD called Heidenhain variant in 1997.

Hutchinson's office did not return a call from UPI.

Eugene Major, who serves as acting director of the NINDS and is responsible for the fate of the brain collection, did not return a call from UPI.

"The patients these brains were taken from suffered greatly before they died of CJD," Heather Larson of Phoenix, whose mother succumbed to CJD last year at the age of 56, wrote in a letter to Arizona Republican Sens. John McCain and Jon Kyl, and Republican Rep. John Shadegg. "Their brains hold answers that can save human lives. Destroying the brains at Bethesda would greatly hinder the research being done to fight this disease and would cost many their lives."

The offices of McCain and Kyl did not return UPI's calls.

"The ravages of this disease, and the toll it takes not only on its victims but on family and loved ones, cannot easily be described to someone who has not witnessed it personally," Patty Cook of Kansas City, Kan., wrote in a letter to Kansas Republican Sens. Sam Brownback and Pat Roberts, and Democratic Rep. Dennis Moore.

"I urge you to do whatever you can to ensure these brains are not destroyed," added Cook, whose mother died of CJD in 1982.

Brownback's office did not return a call from UPI.

CJD belongs to a group of diseases -- called transmissible spongiform encephalopathies or TSEs -- that includes mad cow disease, chronic wasting disease in deer and elk, scrapie in sheep and several types of CJD in humans. There is no cure for CJD and it typically results in death within a year after the onset of symptoms.

Consumer groups also are concerned and are considering taking steps to ensure the brain collection will be preserved.

"This is outrageous," Michael Hansen, a biologist and senior research associate with Consumers Union in Yonkers, N.Y., told UPI. "Those brains are a critical resource for CJD science and they must be at a research facility."

Hansen added that his late friend, Joe Gibbs, the former chief of NINDS's Laboratory of Central Nervous System Studies, told him the brain of famed choreographer George Balanchine, who died of CJD in 1983, resides in the collection.

"How can we claim to be a scientific country if we're going to be throwing away an irreplaceable repository of the first evidence of these diseases?" asked Felicia Nestor, who serves as a consultant to Public Citizen.

There may be hope yet for the collection, however.

Neil Cashman, an expert on TSEs at the University of Toronto's Center for Research in Neurodegenerative Diseases, told UPI he has been attempting to drum up support for acquiring the collection with his colleagues at the University of British Columbia in Vancouver -- where he plans to move this summer.

"I'm trying to organize support for an official letter from UBC to NIH to request the collection," Cashman said.

The letter will probably go out in about a month, he said.

"The goal would be to make it a resource for the world and make the tissues available to scientists who had a reasonable request," he added.

Singeltary said he has heard from at least one other prominent scientist in this field who said they planned to contact the NIH and urge it to reconsider the fate of the collection.

One brain in the collection, that of a French woman who died in 1971, may help provide clues about the origins of variant CJD -- a condition similar to CJD that humans can contract from eating beef products contaminated with the mad-cow pathogen. The first recognized case of vCJD occurred in 1995 in the United Kingdom, but an NIH scientist said he tested the French woman's brain in 2000 and found signs consistent with vCJD -- not CJD.

French researchers currently are re-examining specimens from the case to determine if the woman was indeed infected with vCJD. If she was, it would suggest the disease began infecting people more than 20 years earlier than previously thought.

Cashman said the case underscores the value of the NIH brain collection.

"There is information locked up in these freezers that will be lost forever if this collection is destroyed," he said.

--

E-mail: sciencemail@upi.com

© 2005 United Press International, Inc. All Rights Reserved.


http://www.upi.com/Science_News/2005/04/01/Groups-seek-to-save-NIH-brain-collection/UPI-72961112392131/



NIH sends mixed signals on CJD brains

By Steve Mitchell Medical Correspondent

Washington, DC, Apr. 7 (UPI) -- A National Institutes of Health official who told United Press International the agency might destroy its collection of brains from human patients afflicted with a condition similar to mad cow disease reportedly has told the head of a patient-advocate group the collection would be preserved.

The official, Eugene Major, acting director of the basic neuroscience program at the NIH, has not responded to e-mail or a phone call from UPI seeking clarification of his remarks, and the official status of the collection remains unknown.

As reported by UPI on March 24, the collection is stored in freezers by the NIH's National Institute for Neurological Disorders and Stroke in Bethesda, Md. It contains brains and other tissue samples from hundreds of people who died from the brain-wasting illness Creutzfeldt Jakob disease, as well as tissues from an untold number of experimental animals.

The consensus of scientists in this field is the collection, which dates back to 1963, is invaluable for research and could even provide insight into treatments for the fatal disorder. Currently, there is no cure for CJD and patients typically die within a year after symptoms begin.

Florence Kranitz, president of the non-profit advocacy group CJD Foundation, told UPI she had "a very long conversation" with Major, in which he told her the remaining tissues in the collection would not be destroyed.

"He reassured me in no uncertain terms," Kranitz said, noting constituents of the foundation and other CJD advocacy groups had been expressing concerns to her the tissues would be destroyed.

Kranitz, who has personal reasons for wanting the collection preserved -- her husband died of CJD in 2000 -- said she plans to meet with Major at the end of April to discuss the issue further.

CJD belongs to a group of diseases collectively known as transmissible spongiform encephalopathies, or TSEs, that includes mad cow disease in cows, chronic wasting disease in deer and elk, and scrapie in sheep. All TSEs are incurable and fatal.

Major previously told UPI some samples already have been destroyed and others have been given to researchers at the Food and Drug Administration and the National Prion Disease Pathology Surveillance Center in Cleveland.

Major said the remaining collection "has very little remaining value" and could be destroyed if another entity does not claim them.

Bruce Johnson, a former NIH scientist who retired at the end of 2003, said he had been told the collection would be destroyed in two years if no one took the samples from the NIH.

In response to hearing that Major had failed to confirm to UPI the brain collection would not be destroyed, Patricia Ewanitz, who lives in Port Jefferson Station, N.Y., and is founder of the advocacy group CJD Voice, said, "The brain tissue might not be indispensable to the National Institutes of Health but it is absolutely necessary to the families who thought enough of science to donate the brains, brain tissue and blood in hopes of someday finding an answer to why their loved one died."

Ewanitz, whose husband died of CJD in 1997, added, "It now seems like such a joke."

Terry Singeltary, whose mother passed away from a type of CJD in 1997, said the NIH should use the samples for scientific research, not just store them in freezers.

Both Singeltary and Ewanitz said they would feel more reassured if Major verified in writing the collection will not be destroyed.

"I would go further and ask Major what he plans to do with them," Singeltary said. "If the samples are just going to sit up there and go bad, then they should give them out to researchers looking for cause and cure."

The revelation the NIH might destroy part or all of the collection sparked an outcry from patient advocates, consumer groups and scientists.

Advocates have been contacting their members of Congress, urging them to investigate and prevent the NIH from destroying the brains. Consumer groups also have gotten involved and scientists have taken steps to obtain the collection or have urged Major not to destroy the samples.

Felicia Nestor, who serves as a consultant to Public Citizen, told UPI she had contacted certain legislators and at least one was considering looking into the situation. Nestor asked the legislator's name be withheld.

Kranitz said Major also told her he plans "to advertise in professional neurological journals and by whatever means necessary to make it known" to researchers in the field the tissues are available.

Major previously said, however, that efforts to inform researchers of the availability of the collection were already underway and included informing NIH grantees. He added he had personally notified researchers at scientific meetings, but no TSE researcher contacted by UPI was aware of this.

"I was never informed," said Laura Manuelidis, an expert on these diseases and section chief of surgery in the neuropathology department at Yale University. She said the first she had heard of the situation was in UPI's March 24 report.

Manuelidis also said she contacted Major, expressing interest in the specimens, but so far has not received a response.

"I sent a letter to (Major) on (March 25) about our interest in these specimens, but he has not replied," she told UPI in an e-mail.

Neil Cashman, a TSE expert at the University of Toronto, who said he was not aware the samples might be destroyed, has lobbied colleagues at the University of British Columbia -- where Cashman is scheduled to move to this summer -- to help draft a letter requesting the collection.

The Memorial Institute for Neurodegenerative Diseases Inc., a non-profit organization consisting of more than 40 university and institute researchers from the United States, Canada, the United Kingdom and France, requested the collection in January, 2004. So far, the institute has not been informed of a decision by the NIH.

Asked if Major had told him whether the collection would be preserved, MIND Executive Director Harry Peery said, "We have heard nothing further from Eugene Major or anyone else at the NIH regarding the brain collection."

--

http://www.upi.com/Science_News/2005/04/07/NIH-sends-mixed-signals-on-CJD-brains/UPI-25701112902231/





my letter to Government Officials begging for help ;



-------- Original Message --------

Subject: NIH to destroy our loved ones brain tissues, WE NEED YOUR HELP PLEASE
Date: Fri, 25 Mar 2005 16:04:57 -0600
From: "Terry S. Singeltary Sr."

To: senator@hutchison.senate.gov

CC: Judith.Zaffirini@senate.state.tx.us, Bob.Deuell@senate.state.tx.us, District98.Truitt@house.state.tx.us, District115.Jackson@house.state.tx.us, Jane.Nelson@senate.state.tx.us, District96.Zedler@house.state.tx.us, Jon.Lindsay@senate.state.tx.us, f-gilstrap@tamu.edu, ka-phillips@tamu.edu


References: <422CA640.3030108@wt.net>


Greetings again Honorable Senator Hutchison and other Honorable Members of Texas Office,


My name is Terry S. Singeltary Sr. I lost my Mother to hvCJD aka mad cow.THE Heidenhain Variant of Creutzfeldt Jakob Disease. (there is more than one strain of mad cow disease and i will reference last)

I am once again writing to you on a matter of extreme importance. I would appreciate your assistance in writing to the National Institutes of Health requesting that the brain tissue collected over the years at NINDS from family members of Creutzfeldt-Jakob Disease victims be preserved and recorded and not discarded.

[See attached articles]

THE WASHINGTON TIMES UNITED PRESS INTERNATIONAL

NIH may destroy human brain collection

By Steve Mitchell Medical CorrespondentWashington, DC, Mar. 24 (UPI) -- ...


SNIP...END...TSS



FINALLY ONE, and only one Senator, did reply to my concerns, and help us with preserving the brain tissue bank at NIH. I had sent a hard copy via US postal to the Honorable Senator John Cornyn (see below), but none of the (above) Senators and other officials ever bothered to reply.


See The Honorable John Cornyn Reply below ;




JOHN CORNYN TEXAS UNITED STATES SENATE WASHINGTON, DC 20510-4305 April 26,2005

Mr. Terry Singeltary

P.O. Box 42

Bacliff, Texas 77518

Dear Mr. Singeltary:

In response to your recent request for my assistance, I have contacted the National Institutes ofHealth. I will write you again as soon as I receive a reply. I appreciate having the opportunity to represent you in the United States Senate and to be ofservice in this matter.

Sincerely,

JOHN CORNYN United States Senator JC:djl


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


JOHN CORNYN TEXAS UNITED STATES SENATE WASHINGTON, DC 20510-4305

May 18,2005

Mr. Terry Singeltary

P.O. Box 42

Bacliff, Texas 77518

Dear Mr. Singeltary:

Enclosed is the reply I received from the Department of Health and Human Services in response to my earlier inquiry on your behalf. I hope this will be useful to you. I appreciate having the opportunity to represent you in the United States Senate. Thank you for taking time to contact me. Sincerely,

JOHN CORNYN United States Senate JC:djl Enclosure

DEPARTMENT OF HEALTH & HUMAN SERVICES National Institutes of HealthNational Institute of NeurologicalDisorders and Stroke NINDS Building 31, Room 8A52 31 Center Dr., MSC 2540 Bethesda, Maryland 20892-2540 Phone: 301-496-9746 Fax: 301-496-0296 Email: [log in to unmask]

May 10, 2005

The Honorable John CornynUnited States SenatorOccidental Tower5005 LBJ Freeway, Suite 1150Dallas, Texas 75244-6199

Dear Senator Cornyn:

Your letter to the National Institutes of Health (NIH) forwarding correspondence from Mr. Terry S. Singeltary, Sr., has been forwarded to me for reply. Mr. Singeltary is concerned about thepreservation of Creutzfeldt-Jakob disease (CJD) brain samples that have been maintained by theNational Institute of Neurological Disorders and Stroke (NINDS) Intramural Research programfor many years. I am sorry to learn that Mr. Singeltary's mother died of CJD and can certainly understand hisdesire that any tissues that could help investigators unravel the puzzle of this deadly disease arepreserved. I hope he will be pleased to learn that all the brains and other tissues with potential tohelp scientists learn about CJD are, and will continue to be, conserved. (The tissues that arediscarded are those that have either decayed to an extent that renders them no longer appropriatefor research or those for which we do not have sufficient identification.) The purpose of gathering these brains and tissues is to help scientists learn about CJD. To that end, some of the NINDS-held samples are distributed to investigators who can demonstrate thatthey have a compelling research or public health need for such materials. For example, sampleshave been transferred to NIH grantee Dr. Pierluigi Gambetti, who heads the National PrionDiseases Pathology Surveillance Center at Case Western Reserve University in Ohio and workswith the Centers for Disease Control and Prevention to monitor all cases of CJD in the UnitedStates. Dr. Gambetti studies the tissues to learn about the formation, physical and chemicalproperties, and pathogenic mechanisms of prion proteins, which are believed to be involved inthe cause of CJD. Samples have also been transferred to Dr. David Asher, at the U.S. Food andDrug Administration, for use in assessing a potential diagnostic test for CJD.

Page 2 - The Honorable John Cornyn

in closing, we know that donating organs and tissue from loved ones is a very difficult andpersonal choice that must often be made at the most stressful of times. We at the NINDS aregrateful to those stalwart family members who make this choice in the selfless hope that it willhelp others afflicted with CJD. We also know the invaluable contribution such donations maketo the advancement of medical science, and we are dedicated to the preservation of all of thetissue samples that can help in our efforts to overcome CJD.

I hope this information is helpful to you in responding to Mr. Singeltary. Sincerely,

Story C. Landis, Ph.D. Director, National Institute ofNeurological Disorders and Stroke


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


NIH says it will preserve CJD brains

By STEVE MITCHELL

WASHINGTON, May 31 (UPI) -- The National Institutes of Health apparently has reversed its position on the fate of an invaluable collection of brains from people afflicted with a condition similar to mad cow disease, saying in a letter to a U.S. senator it will not destroy the collection.

An NIH official had told United Press International previously that the brain collection, which consists of samples from hundreds of people who died from the brain-wasting illness called Creutzfeldt Jakob disease, could be discarded if another entity does not claim them.

That sparked an outcry from patient-advocacy groups, consumer watchdogs and scientists, and the agency now appears to have backed away from that course.

"All the brains and other tissues with potential to help scientists learn about CJD are, and will continue to be, conserved," Story Landis, director of the National Institute of Neurological Disorders and Stroke, which oversees the brain collection, wrote in a May 10 letter to Sen. John Cornyn, R-Texas.

Cornyn had inquired about the status of the collection in April.

Last March, Eugene Major, acting director of the basic neuroscience program at the NIH, told UPI the useful portions of the collection had been doled out to scientists and the remaining samples had "very little remaining value" and could be destroyed.

Landis could not be reached for comment Tuesday. NINDS spokesman Paul Girolami told UPI he had been unable to locate her.

Scientists think the collection, which dates back to 1963, is invaluable for research on CJD and similar diseases and could even provide insight into treatments. There is no cure for CJD and patients typically die within a year after symptoms begin.

"Absolutely, the collection is worth keeping," Bruce Johnson, a former NIH scientist who said he had been told the collection would be destroyed in two years if no one took the samples from the agency, told UPI.

The Memorial Institute for Neurodegenerative Diseases Inc., a non-profit organization consisting of more than 40 researchers from several countries, offered to take the collection off of NIH's hands more than a year ago and so far has not heard anything from the agency, Harry Peery, MIND's executive director, told UPI.

CJD belongs to a group of incurable and fatal diseases collectively known as transmissible spongiform encephalopathies, or TSEs, that includes mad cow disease in cows, chronic wasting disease in deer and elk, and scrapie in sheep.

Variant CJD, or vCJD, is a relatively new TSE, which people can contract from consuming beef products infected with the mad cow pathogen.

Despite Landis' assurance the collection will be preserved, some family members of the patients who donated their brains to the NIH are still skeptical. This is because the wording Landis used in the letter leaves open the possibility that some brain samples are being destroyed.

"The tissues that are discarded are those that have either decayed to an extent that renders them no longer appropriate for research or those for which we do not have sufficient identification," Landis wrote.

"Which ones" are being destroyed? asked Terry Singeltary, who is involved with several CJD patient groups.

"With a system like this, they could destroy whatever and whenever they wanted, for whatever reason they wanted," Singeltary, whose mother died of CJD in 1997, told UPI.

"It's a perfect excuse to discard some suspicious tissue resembling vCJD or some atypical TSE related to animal TSEs in the USA," he added.

Although the collection includes samples from CJD patients as young as 16 that could make them candidates for possible vCJD, the brains have never been screened for evidence of the disease. The only confirmed vCJD case in the United States occurred in a Florida woman who is thought to have contracted the disease in England.

Johnson said he along with renowned CJD expert Paul Brown were in the process of sorting through the samples to match them up with patient identification documents until they both retired. Some of the samples may prove impossible to identify, he said, but he and Brown are the only ones familiar enough with the collection to organize it and neither has been asked back by the agency to aid in the identification process.

Steve Mitchell is UPI's Medical Correspondent. E-mail: [log in to unmask]

Copyright 2005 by United Press International. All Rights Reserved.


http://www.upi.com/Science_News/2005/05/31/NIH-says-it-will-preserve-CJD-brains/UPI-67711117574761/




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


US scientist accused of selling tissue samples Deal said to earn $285,000 for vials that cost millions By Diedtra Henderson, Globe Staff June 14, 2006

WASHINGTON -- A senior government scientist pocketed hundreds of thousands of dollars as a drug company consultant in exchange for human tissue samples that cost the federal government millions to acquire, congressional investigators said yesterday .

The House Energy and Commerce Committee report, the culmination of a one-year inquiry, was released hours before a two-day hearing began to explore the government's practices for procuring human tissue samples. According to congressional investigators, the National Institutes of Health's Dr. Trey Sunderland agreed to collaborate with Pfizer Inc. , the world's largest drug company. Sunderland, chief of the geriatric psychiatry branch of the National Institute for Mental Health , sent Pfizer 3,200 tubes of spinal fluid and 388 tubes of plasma collected for Alzheimer's research.

The government spent $6.4 million to obtain the 3,500 samples that showed how Alzheimer's disease progressed in 538 subjects.

Pfizer paid Sunderland $285,000 in consulting fees related to the samples, investigators said. In total, Pfizer paid him more than $600,000 from 1998 to 2004 for outside consulting and speaking fees. Sunderland is scheduled to testify today at the hearing.

``Contrary to the House committee report, Dr. Sunderland did not receive any payments from Pfizer for human tissue samples," said Robert F. Muse, the scientist's Washington, D.C., attorney. ``He acted properly, ethically, and legally in his relationship with Pfizer."

Pfizer spokeswoman Kate Robins said the company had a transfer agreement endorsed by the NIH that permitted Sunderland to send cerebrospinal fluid from research participants with Alzheimer's, the participant's relatives who were at higher risk of developing the neurological disease, and elderly adults with normal Alzheimer's risk.

Sunderland's consulting role tapped his Alzheimer's disease expertise to look for signals in the samples that could help identify and diagnose the disease.

``The payments over a six-year period were reasonable and customary for an expert of Dr. Sunderland's stature, and reflect the fair-market value of his consulting services," Robins said.

The report said the tissue transfers, reported by a government whistleblower, raised serious questions about how the government ensures its scientists do not abuse their positions and about the agency's ability to track the valuable samples.

``NIH tells us it has no centralized inventory system that could tell the NIH director how many vials of tissues are in freezers at a particular institute," said Representative Joe Barton , Republican of Texas and House Energy and Commerce Committee chairman . ``It would really be a shame if we find out that the National Institutes of Health has more control over its paper clips and trash cans than it has over its human tissue samples."

John T. Burklow , a NIH spokesman, said the agency shares ``the committee's concerns in regard to the ethical management of human tissue samples."

Sunderland's arrangement with the drug maker -- made without NIH knowledge, according to Burklow -- occurred after the agency relaxed its ethics policy covering scientists' outside activities and ended before the agency enacted more stringent rules.

The NIH, pressured by Barton's committee, on Aug. 25 curbed outside consulting deals between its scientists and pharmaceutical and biotechnology companies.

Diedtra Henderson can be reached at [log in to unmask]

© Copyright 2006 Globe Newspaper Company.


http://www.boston.com/business/globe/articles/2006/06/14/us_scientist_accused_of_selling_tissue_samples/



NIH Scientist Exploited Human Tissue Samples For Personal Gain, Report Finds

Academic Shipped 3,500 Tubes to Pfizer; Pocketed $285,000

WASHINGTON - A senior scientist at the National Institutes of Health (NIH) shared thousands of valuable human tissue samples with the drug giant Pfizer, netting himself at least $285,000 and raising serious questions about existing government safeguards against abuse, a new House Energy and Commerce Committee staff report has found.

The report was released today in conjunction with a two-day Subcommittee on Oversight and Investigations hearing entitled "Human Tissue Samples: NIH Research Policies and Practices."

According to the committee's investigation, Dr. Trey Sunderland, chief of the Geriatric Psychiatry Branch of the National Institute for Mental Health, shipped 3,200 tubes of spinal fluid and 388 tubes of plasma collected for Alzheimer's research. As a result, Dr. Sunderland was paid $285,000 by Pfizer for consulting work related to the samples. All told, Dr. Sunderland received more than $600,000 in payments from Pfizer from 1998 to 2004 for outside consulting and speaking with any record of prior approval or disclosure in his government financial report filings.

Scientists considered the 3,500 samples, chronicling the progression of Alzheimer's disease on the same subjects over several years, to be priceless. An analysis by committee staff determined that simply procuring the samples from 538 subjects cost U.S. taxpayers $6.4 million. "It was unlikely that anywhere but at the clinics of NIH could this unique historical collection of human tissue samples be assembled," the report reads.

Committee investigators concluded that there were "reasonable grounds to believe" that obtaining the samples was a "primary reason for Pfizer's interest in collaborating with Dr. Sunderland."

The NIH's Office of Management Assessment has found that Dr. Sunderland's misconduct violated ethics rules as well as federal law and regulation. Yet, years after the transactions, he has not been prosecuted or disciplined to any measurable degree. He remains an employee at NIH and a member of the Public Health Service Corps.

The committee's year-long inquiry into the collections, storage, tracking and use of human tissue samples in the NIH's intramural research program was prompted in part by concerns raised by a former NIH scientist, Dr. Susan Molchan, in April 2005.

Human tissues include everything from DNA to organs to blood and they play a critical role in modern scientific research. According to the RAND Corporation, there are more than 307 million tissue samples from more than 178 million people stored in the United States .

Among the report's other findings:

The National Institutes of Health have "no uniform, centralized, and mandatory authority regulating the handling of human tissue samples. Some NIH laboratories kept a written record on the maintenance of these samples, but other NIH laboratories did not." NIH had "no formal inventory control or tracking system. ... Moreover, the lack of accountability left NIH wholly vulnerable to theft and diversion of valuable human tissue samples. These preliminary inquiries raised serious concerns over what was described to committee staff by NIH officials of a fairly loose, ad-hoc approach to controlling human tissue samples." http://energycommerce.house.gov/108/News/06132006_1941.htm

Barton: NIH Must Regain Control of Tissue Samples, Ethical Lapses 'It would really be a shame if we find out that the National Institute of Health has more control over its paper clips and trash cans than it has over its human tissue samples.' - Chairman Joe Barton

WASHINGTON - U.S. Rep. Joe Barton, R-Texas, chairman of the House Energy and Commerce Committee, issued the following statement today as part of an Oversight and Investigations Subcommittee hearing entitled "Human Tissue Samples: NIH Research Policies And Practices:"

"As I said, at the last set of oversight hearings on NIH, the hallmark of this committee has always been its oversight responsibility and its willingness and ability to hold agencies responsible under its jurisdiction and that produce results in better government and better services for the American people.

"Two years ago, we found that there were weaknesses in the system at that time and that those weaknesses were more severe than had been previously recognized. To his credit, Dr. Zerhouni, had a ringside seat at these hearings and took the facts of the hearings seriously and changed and reformed the NIH ethics system.

"Today we are going to take a look at how NIH protects its most precious assets -- the material that is at the core of the NIH research mission: human tissue samples. Once again, after extensive investigation on a bipartisan basis, we have found deeper concerns regarding human tissue samples at NIH than we first believed. We have found a lack of a centralized database and a lack of oversight at NIH that could, and probably does, leave NIH laboratory vulnerable to the risks of theft and abuse. We know from previous investigations that NIH has an inventory system, but NIH tells us it has no centralized inventory system that could tell the NIH director how many vials of tissues are in freezers at a particular institute. It would really be a shame if we find out that the National Institute of Health has more control over its paper clips and trash cans than it has over its human tissue samples.

"The committee has investigated a case and found evidence of a serious breach of trust. This case has focused on Dr. Trey Sunderland, who is supposed to be a witness later in this hearing. He's a very noted and respected researcher in the field of Alzheimer's disease. I wish we were here to discuss some great discovery he made to cure, or at least alleviate, the hazards of Alzheimer's. Instead, we are discussing the ways he used his position to use NIH spinal fluid samples to further his undisclosed, personal consulting. The information provided to the committee shows that Pfizer paid Dr. Sunderland $285,000 during the 1998-2003 time period to consult on two projects involving spinal fluid samples that Dr. Sunderland sent to Pfizer. During this same time period, Pfizer also paid Dr. Sunderland approximately $300,000 for lectures. These figures don't even count almost $200,000 additional for undisclosed activities with other companies. There is also evidence that he advised his subordinate to conceal these consulting activities involving the samples. This is from an official who for 10 years chaired a committee that reviews the ethics of conducting mental health research on human beings. This certainly appears to be a betrayal of the public trust that NIH stands for.

"These hearings underscore the need to enact NIH reauthorization and reform legislation. The NIH director must have some baseline of information about NIH assets. If we are going to gain new efficiencies and hopefully more effective ways to translate research into better healthcare, enacting NIH reauthorization legislation is of the highest importance.

"Out of this investigation of disturbing questions and concerns, we can use these hearings to make NIH stronger and better. The National Institute of Health is indeed a national treasure. It must be cherished, protected, nourished and allowed to flourish. Today's hearings are a first step toward strengthening public trust in NIH research and preserving confidence in its integrity.

"I thank you, Mr. Chairman, and Mr. Stupak for the bipartisan nature of this investigation. I also thank Mr. Dingell for his support of this investigation. Finally, I look forward to working with Dr. Zerhouni and the leadership of NIH on this matter and helping NIH become better managed -- and thus deliver the results for the health of America that we so depend on NIH to do."

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http://energycommerce.house.gov/108/News/06132006_1949.htm



FOR IMMEDIATE RELEASE Tuesday, June 13, 2006

CONTACT: OD Office of Communications and Public Liaison 301-496-5787

NIH Statement Regarding House Hearing on Human Tissue Samples

Attribution: John Burklow, NIH spokesman

NIH’s position on ethics is clear: any conflict of interest resulting in an individual personally profiting from official government research activities cannot be tolerated. We are committed to maintaining the public’s trust in NIH and its scientists as an unbiased source of biomedical research guidance and advice. The case under consideration concerns events that began in 1998 — after the NIH ethics rules concerning outside activities were relaxed — and that ended before the new rules were put in place. NIH has previously referred this case to the relevant authorities for appropriate action.

It is important to note that the specific consulting arrangements in question, had they been known to NIH, would not have been approved under the present or previous ethics regulations. Outside consulting connected to an NIH employee’s official government duties has always been prohibited at NIH.

NIH has undertaken a comprehensive review of its activities and conflict of interest policies in the last few years. As a result of that process, on August 25, 2005, NIH implemented comprehensive ethics rules that make it clear what NIH scientists can and cannot do in regard to outside activities. These new rules removed any ambiguity about what is allowed or not allowed. Here are two important points:

Under new NIH regulations, all NIH employees are now prohibited from engaging in outside employment with pharmaceutical companies and biotechnology companies in their private capacities — period. Collaboration and partnership with industry can nonetheless be very valuable in scientific pursuits and NIH rules allow such activities, as long as they are undertaken through an officially approved Cooperative Research and Development Agreement (CRADA). Although we cannot discuss this particular case because it remains under investigation, we can speak to the relevant issues that it raises.

Collaborations among scientists that involve human tissue samples are common and essential for science. There are, however, stringent rules in place to protect the participants who donated their samples, and to ensure that there is full informed consent.

We share the Committee’s concerns in regard to the ethical management of human tissue samples and the development of rigorous and uniform policies to protect the public’s trust and interests, while advancing science to address important public health problems. The thousands of scientists who work at NIH have always been and remain committed to these principles.

The Office of the Director, the central office at NIH, is responsible for setting policy for NIH, which includes 27 Institutes and Centers. This involves planning, managing, and coordinating the programs and activities of all NIH components. The Office of the Director also includes program offices which are responsible for stimulating specific areas of research throughout NIH. Additional information is available at http://www.nih.gov/icd/od/.

The National Institutes of Health (NIH) — The Nation's Medical Research Agency — includes 27 Institutes and Centers and is a component of the U.S. Department of Health and Human Services. It is the primary federal agency for conducting and supporting basic, clinical and translational medical research, and it investigates the causes, treatments, and cures for both common and rare diseases. For more information about NIH and its programs, visit www.nih.gov.


http://www.nih.gov/news/pr/jun2006/od-13.htm



THE LEGALITY OF STEALING ORGAN/TISSUE...TEXAS STATUTES


http://creutzfeldt-jakob-disease.blogspot.com/2008/01/cjd-hgh-body-snatchers.html



Friday, August 07, 2009

CJD Human Cornea Tissue, Recall END OF ENFORCEMENT REPORT FOR AUGUST 5, 2009


http://creutzfeldt-jakob-disease.blogspot.com/2009/08/cjd-human-cornea-tissue-recall-end-of.html





NATIONAL CREUTZFELDT-JAKOB DISEASE SURVEILLANCE UNITSCIENTIFIC REPORT2007/08


NCJDSU Scientific Report 2008

SPORADIC CJD

Question 7. What is the cause of sporadic CJD (sCJD) ? What are the risks of secondary transmission of sCJD?

Case control studies

Risk factor information has been collected for cases of sporadic CJD since before the Unit was established in 1990. Over the years various control groups have been recruited; the method chosen depending on the resources available and anticipated validity. These have been detailed in various previous NCJDSU Annual Reports.

Since 1990 there have been five papers published examining risk factors for sCJD that the Unit has either led or collaborated in:-

1) Wientjens et al, Neurology 1996. A meta analysis of three case control studies (178 CJD cases and 333 controls). The results showed an elevated risk of CJD for those with a family history of dementia, a history of poliomyelitis, those employed as health professionals and those exposed to cows and sheep. There was no association with consumption of animal organs, including brain.

2) van Duijn et al, Lancet 1998- this compared 405 CJD cases and 405 hospital controls recruited as part of the 1993-95 EU collaborative studies of CJD in Europe. The findings suggested that genetic factors other than CJD mutations may play an important part in CJD. Iatrogenic transmission seemed rare in the population studied. There was little evidence of association between the risk of CJD and animal exposure or consumption of processed bovine meat or milk products for the period studied.

3) Zerr I et al, J Clin Epid 2000- medical risk factors were examined using the 405 CJD cases and 405 hospital controls recruited as part of the 1993-95 EU collaborative studies of CJD in Europe. The study failed to identify any common medical risk factor for CJD.

4) Ward et al, Neurology 2002- Surgical risk factors from 326 sCJD cases recruited as part of the 1993-95 EU collaborative studies of CJD in Europe were compared with 326 community controls recruited by telephone in 2000. A history of surgery was associated with risk of sCJD and the results supported the hypothesis that sCJD may result from hitherto unrecognised surgical contamination events.

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NCJDSU Scientific Report 2008

5) Ward et al, Annals of Neurology 2007- Medical risk factors for 431 sCJD cases resident in the UK and referred to NCJDSU between 1998-2006 were compared with 454 general population control subjects recruited 2002-2003 (see NatCen controls in Appendix 6). This study found some evidence for a link between increased risk of sCJD and surgery, however there was no convincing evidence of temporal-geographical links between cases undergoing neurosurgery or gynaecological surgery. It concluded that it was unlikely that a high proportion of UK sCJD cases were the result of surgical transmission, but the possibility of such transmission cannot be excluded.

As for vCJD (see Questions 2 and 5 above), further work examining data from general practitioner records of cases and controls needs to be carried out in order to accurately determine risk for sCJD associated with medical or surgical procedures. This is on-going, though the priority has been given to vCJD at present.

Blood transfusion

An analysis of the potential for blood transfusion to be a risk factor for the development of sporadic CJD has been undertaken collaboratively by the major EU countries (coordinator M Pocchiari). A prospective study is under consideration.

Genetic factors

Possible genetic factors related to sCJD are being investigated by the Unit, both internally and through external collaboration (see Question 8 below).

Geographical distribution

The investigation of the geographical distribution of sporadic CJD by genetic and molecular subtype is on-going in the Unit.

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NCJDSU Scientific Report 2008

Question 8: What are the clinico-pathological, genetic & molecular features of sporadic CJD and how are they related?

While this is an interesting question in its own right, it has an extremely important bearing on the Unit’s core surveillance function, particularly in relation to vCJD. This is because the most important and potentially difficult differential diagnosis of vCJD is sCJD; many initial reports of suspect cases of vCJD in the UK and elsewhere, have been found to be atypical cases of sCJD. A full characterisation of the clinico- pathological, epidemiological and molecular phenotypes of sCJD is therefore clearly essential, which the Unit has continued to carry out within the UK.

Atypical clinical presentations, disease courses and pathological findings are found in only a small percentage of sCJD cases and so are difficult to characterise, even in the UK population of 50-60 million. This is of particular importance in relation to any attempt to recognise new clinical disease phenotypes either related to BSE or, potentially, to other animal diseases. Therefore, our international collaborations have contributed greatly to the analysis of clinical, epidemiological and pathological data. For example, within the NEUROCJD collaboration, a detailed study was undertaken of particular presentations of sporadic CJD, such as pure cerebellar ataxia and Heidenhain’s syndrome (currently being prepared for publication). Studies of atypical forms and cases with young age of onset are in progress (Murray et al, J Neurol Neurosurg Psychiatry 2008). The large number of cases of sCJD accumulated within the system allowed a detailed study of the factors that separately influence disease duration (Pocchiari et al, Brain 2004).
It is well established that the clinico-pathological features of sCJD vary with PRNP-129 genotype and PrP protein type. Both the Unit’s Molecular Genetic and Protein Laboratories contribute to the full clinico-pathological-molecular characterisation of sCJD cases and research is being undertaken into these correlations. Research into possible genetic factors that affect susceptibility and disease phenotype in sCJD has been described above in Question 3.
The differentiation of atypical sCJD from vCJD is potentially aided by prion protein molecular data. However, the relationship between PrP protein type and CJD strain is not as straightforward as it initially seemed and, in particular, the Unit has been active in research into the phenomenon of the co-occurrence of prion types in individual cases of

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NCJDSU Scientific Report 2008

CJD. Work carried out by the Prion Protein Laboratory used a type 1 specific antibody (12B2) to show that type 1 PrPres is a minor component in brains of sporadic CJD cases previously classified as type 2 and also that a minority type 1 component is present in BSE brain, in all tested cases of vCJD and in vCJD transmitted to wild-type mice (Yull et al, Am J Pathol 2006). Whilst this work provoked interest and a study with similar conclusions was published by the Aguzzi laboratory (University Hospital, Zurich), the findings are somewhat controversial. To address this, the Unit has conducted a detailed study of the WHO CJD standard reference materials (available from the UK National Institute for Biological Standards and Control) to compare the PrPres mixtures in cases of sporadic CJD that are acknowledged to be genuine mixtures, to those of vCJD where antibodies such as 12B2 are needed to detect the type 1 component. A paper is in preparation for publication. The distribution of disease-related prion protein in extra-neural tissues (such as skeletal muscle and pituitary) in cases of sCJD is an area of developing interest (Peden et al, Am J Pathol 2006; J Gen Virol 2007), which the Prion Protein Laboratory plans to continue to pursue in the future.

The protein laboratory studies have also included work on other forms of CJD. The study of iatrogenic CJD shows the presence of types 1 and/or type 2 PrPres, similar to those found in sporadic CJD, but with a very different protein type and codon 129 genotype distribution. These data, and that on panencephalopathic CJD, have been correlated with clinico-pathological data and are currently being prepared for publication and provide valuable comparisons with the sporadic and variant forms of CJD.

The relationship between PrPres type and agent strain is being investigated by ongoing analysis of transmission to wild-type mice in collaboration with the Neuropathogenesis Division, Roslin Institute. Studies of the transmission characteristics of subtypes of sporadic CJD in a human transgenic model have also been completed (J Manson) and provide important information on the extent of strain variation in sCJD and the influence of codon 129 genotype and prion protein type. This later project is harmonised with the EU funded HUMTRANS project which aims at identifying strain variation in all forms of human prion disease, including ’atypical’ cases (J Manson).

Complementing animal transmission studies the Prion Protein Laboratory aims to model the transmission of human prions, and prion protein conversion, by comparing the

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NCJDSU Scientific Report 2008

results of mouse transmission studies with the infection of cell cultures (including human stem cells) and the cell-free protein misfolding cyclic amplification (PMCA) method. The results of initial studies using PMCA indicate that amplification depends on both host and agent factors and that PrPres types are amplified with fidelity from sCJD brain (Jones & Head, unpublished observation). ...


snip...


http://www.cjd.ed.ac.uk/scientificreport.pdf




also see ;





Further characterisation of the prion protein molecular types detectable in the NIBSC CreutzfeldteJakob disease brain reference materials


1.4. Mixed PrP types The co-occurrence of types 1 and 2 in cases of sCJD is now a well recognised phenomenon [5,10,11,12] and several independent studies have each concluded that when an extensive brain sampling protocol is employed 20-50% of sCJD cases can be seen to contain both type 1 and type 2 PrPres [7,10,13,14,15].


AMAZING !!!

terry




Further characterisation of the prion protein molecular types detectable in the NIBSC CreutzfeldteJakob disease brain reference materials

Helen M. Yull, James W. Ironside, Mark W. Head* National CJD Surveillance Unit, School of Molecular & Clinical Medicine (Pathology), University of Edinburgh, Edinburgh, United Kingdom

Received 17 November 2008; revised 29 December 2008; accepted 23 January 2009

Abstract

Sporadic and variant CreutzfeldteJakob disease brain reference materials available from the UK National Institute for Biological Standards and Control have been subjected to further characterisation by Western blot analysis, with particular reference to the co-occurrence of different abnormal disease-associated prion protein (PrPSc) types. The results confirm the presence of genuine type 1 and type 2 protease-resistant PrP (PrPres) in each of the three sporadic CreutzfeldteJakob disease reagents, and provide evidence supporting the lower level presence of type 1 PrPres in the variant CreutzfeldteJakob disease reagents. We conclude that these reagents provide a valuable resource for future research and development.


snip...


1.4. Mixed PrP types The co-occurrence of types 1 and 2 in cases of sCJD is now a well recognised phenomenon [5,10,11,12] and several independent studies have each concluded that when an extensive brain sampling protocol is employed 20-50% of sCJD cases can be seen to contain both type 1 and type 2 PrPres [7,10,13,14,15].

snip...


On a superficial level the presence of more than one PrPSc type in individual CJD brains may seem at variance with the molecular strain typing hypothesis, which proposes that individual prion strains are enciphered by unique and self-perpetuating conformations and glycosylation states. However, this is not necessarily the case. It has long been known that multiple strains may be derived in mice from individual scrapie isolates, and cross-species transmission can, on occasion lead to an abrupt change in apparent strain characteristics.


snip...see full text ;


2009 The International Association for Biologicals. Published by Elsevier Ltd. All rights reserved. Keywords: CreutzfeldteJakob disease; Standards; Prion protein; Molecular typing; Co-occurrence



http://www.sciencedirect.com/science?_ob=ArticleURL&_udi=B6WBS-4VRWNKF-3&_user=10&_rdoc=1&_fmt=&_orig=search&_sort=d&_docanchor=&view=c&_acct=C000050221&_version=1&_urlVersion=0&_userid=10&md5=5a0c2f13b5476b0ddfea5bf9882300df




Beyond PrPres Type 1/Type 2 Dichotomy in Creutzfeldt-Jakob Disease

ArticleRelated ContentComments: 0.Formal Correction: This article has been formally corrected to address the following errors.

To add a note, highlight some text. Hide notes Make a general comment Jump to

Abstract Author Summary Introduction Materials and Methods Results Discussion Author Contributions References Emmanuelle Uro-Coste1#, Hervé Cassard2#, Stéphanie Simon3, Séverine Lugan2, Jean-Marc Bilheude4, Armand Perret-Liaudet5, James W. Ironside6, Stéphane Haik7,8, Christelle Basset-Leobon1, Caroline Lacroux2, Katell Peoch'9, Nathalie Streichenberger5, Jan Langeveld10, Mark W. Head6, Jacques Grassi3, Jean-Jacques Hauw8, Francois Schelcher2, Marie Bernadette Delisle1, Olivier Andréoletti2*

1 INSERM U858, Institut de Médecine Moléculaire de Rangueil and Service d'Anatomie Pathologique et Histologie-Cytologie, C.H.U. Rangueil, Toulouse, France, 2 UMR Institut National de la Recherche Agronomique (INRA)/Ecole Nationale Vétérinaire de Toulouse (ENVT) 1225, Interactions Hôtes Agents Pathogènes, ENVT, Toulouse, France, 3 Commissariat à l'Energie Atomique (CEA), Service de Pharmacologie et d'Immunologie, DRM, CEA/Saclay, Gif sur Yvette, France, 4 Bio-Rad, Research and Development Department, Marnes-la-Coquette, France, 5 Hôpital Neurologique, Services de Neurochimie et de Pathologie, Bron, France, 6 National Creutzfeldt-Jakob Disease Surveillance Unit, Division of Pathology, University of Edinburgh, Western General Hospital, Edinburgh, United Kingdom, 7 INSERM, Equipe Avenir, Maladies à Prions chez l'Homme, Paris, France, 8 Neuropathology Laboratory, Salpêtrière Hospital, AP-HP, Paris, France, 9 Service de Biochimie et Biologie Moléculaire, Hôpital Lariboisière, Paris (Laboratoire associé au CNR “ATNC”) et EA 3621 Faculté de Pharmacie, Paris, France, 10 Central Institute for Animal Disease Control CIDC-Lelystad, Lelystad, The Netherlands

Abstract Top Sporadic Creutzfeldt-Jakob disease (sCJD) cases are currently subclassified according to the methionine/valine polymorphism at codon 129 of the PRNP gene and the proteinase K (PK) digested abnormal prion protein (PrPres) identified on Western blotting (type 1 or type 2). These biochemically distinct PrPres types have been considered to represent potential distinct prion strains. However, since cases of CJD show co-occurrence of type 1 and type 2 PrPres in the brain, the basis of this classification system and its relationship to agent strain are under discussion. Different brain areas from 41 sCJD and 12 iatrogenic CJD (iCJD) cases were investigated, using Western blotting for PrPres and two other biochemical assays reflecting the behaviour of the disease-associated form of the prion protein (PrPSc) under variable PK digestion conditions. In 30% of cases, both type 1 and type 2 PrPres were identified. Despite this, the other two biochemical assays found that PrPSc from an individual patient demonstrated uniform biochemical properties. Moreover, in sCJD, four distinct biochemical PrPSc subgroups were identified that correlated with the current sCJD clinico-pathological classification. In iCJD, four similar biochemical clusters were observed, but these did not correlate to any particular PRNP 129 polymorphism or western blot PrPres pattern. The identification of four different PrPSc biochemical subgroups in sCJD and iCJD, irrespective of the PRNP polymorphism at codon 129 and the PrPres isoform provides an alternative biochemical definition of PrPSc diversity and new insight in the perception of Human TSE agents variability.

snip...

Discussion Top Coexistence of Different PrPres Types in the Same Subject In this study, detection, by WB, of the coexistence of two PrPres types in about 30% (13/41) of cases is consistent with already published data [12],[14]. This observation could suggest the existence in brain from a single patient of different abnormal PrP species. Although two main PK cleavage sites are associated with PrPres type 1 and type 2 (respectively amino acid 82 and 97), N-terminal sequencing revealed in all investigated cases the presence of a whole spectrum of overlapping cleavage sites. Moreover in a part of investigated cases this technique demonstrated the presence (i) of variable but consistent level of type 1 PrPres in patients classified type 2 using WB and (ii) in some patient classified type 1, of low amount of type 2 PrPres [10]. These observations could suggest that, rather than a pure type 1 or type 2 PrPres, PK digestion of a PrPSc specific conformer generate variable mixture of PrPres fragments (with presence of dominant or sub dominant type 1 or type 2 PrPres), which WB usually failed to reveal accurately because its intrinsic technical limits [14]. Antibodies either harbouring higher affinity to PrP (like Sha31) [18] or probing specifically type 1 PrPres (like 12B2) [20], now allow a better perception of such mixture. However, investigations carried out using artificial mixture of type 1 and type 2 brain homogenate, even using high affinity anti-PrP antibodies, clearly indicate the current limits of WB discriminative power [14]. Together, these data suggest that WB analysis of PrPres on its own could be misleading for adequate discrimination between PrPSc variants in CJD.

Both PrPSc PK resistance ELISA and strain typing ELISA are based on the characterization the N terminal part of the PrPSc PK digestion either by increasing PK amount or modifying detergent conditions. While WB profile could be compared to a snapshot picture of PrPres fragments generated by PK digestion process, these assays reflect the dynamics of the PK cleavage rather than its final result (different forms of PrPres). Consequently they could provide different but also more accurate perception of the PrPSc conformers.

Our findings from the PrPSc capture immunoassays clearly indicate that in a single patient, irrespective of brain area, sCJD associated PrPSc displays uniform biochemical properties, regardless of the regional variation of type 1 and type 2 isoforms determined by WB. Such findings support the idea of the presence of a specific TSE agent in each brain and the accumulation of a single associated PrPSc conformer.

sCJD Classification Because the limited size of our cohort of cases, an in depth comparison between the PrPSc signature (as established in this study) and the Parchi classification system is not possible.

However, despite this limitation, two major groups were identified in our panel according to the PrPSc properties. The first major group was constituted with patients harbouring a highly PK resistant PrPSc (MM1 and MV1 patients). The second group included patients harboring a PK labile PrPSc (VV2 and MV2 patients). Using both lesion profile and clinical parameters [2], two major forms of sCJD are commonly recognized. The first sCJD form, named “classical”, is characterized by a “rapid evolution” (usually around 4 months), and affects most of the MM1 and MV1 patients. The second sCJD form, named “atypical”, affects VV2 and MV2 with a longer symptomatic evolution (usually longer than 6 months) and a late dementia. Despite inter-individual variations, sCJD Groups 1 and 2, as we defined them on biochemical criteria were consistent with this classification.

Both VV1 and MM2 sCJD cases are extremely rare; they respectively represent 1% and 4% of the identified sCJD cases. According to the literature, these patients have clinical features and lesion profiles that are very different from other sCJD patients [2]. However, in our study as in previously published studies, WB did not identify any distinct biochemical difference from other type 1 and type 2 cases. In contrast, both the strain typing ELISA and PrPSc resistance assays clearly differentiated these cases from Group 1 and Group 2 cases. This finding, which is consistent with clinico/pathological observations carried out in patients, could indicate that there are indeed differences in PrPSc that distinguish these VV1 and MM2 cases from other sCJD groups.

Prion Strains and PrPSc Phenotype Although prion strains can only be identified definitively by bioassay, molecular in vitro tools to characterize PrPSc are more and more widely used for the rapid identification of particular agents, such as BSE in cattle, sheep, rodent and humans (vCJD) [20],[21]. This has come to be termed “molecular strain typing” and although widely employed, the exact relationship between PrPSc biochemistry and the biological properties of the agents responsible remain to be determined. In sCJD, the presence of four distinct PrPSc biochemical forms apparently correlated to clinico-pathological phenotypes as defined by Parchi et al. [2] could be an indication of the involvement of different TSE agents.

iCJD cases are a consequence of accidental human to human TSE transmission, most likely representing transmission of sCJD. The identification in iCJD cases of the four PrPSc signatures identified in sCJD is consistent with the existence of distinct prions associated with these biochemical forms.

Three examples of human-to-human transmission of variant CJD through blood transfusion have now been identified. While all blood donors were MM at codon 129 PRNP, the recipients had either a MM (n = 2) or a MV genotype (n = 1). Despite this genotype difference there appears to have been conservation of the disease phenotype and PrPres type in all “secondary” vCJD cases [22]–[25]. These observations could suggest that in case of inter-human transmission, difference in donor/recipient genotype could result in un-altered abnormal PrP signature.

Our identification of MM GH iCJD cases harbouring similar PrPSc signature as a VV1 sCJD case or of a VV dura mater iCJD case similar to MM2 sCJD might indicate preservation of a specific PrPSc biochemical signature after human to human transmission between individuals of different codon 129 genotypes.

Treatment with extracts of GH contaminated by CJD has lead to a high number of iCJD cases in France and the UK. The codon 129 genotypes of the affected individuals in the two countries differ, with the French cohort predominantly MM and MV and the British cohort MV and VV [26]. In the absence of any clear explanation for this finding, it was suggested that it might be due to contamination of different batches of GH with different prion strains from individuals of differing PRNP codon 129 genotypes. Our identification of different biochemical forms of PrPSc in GH French patients and in UK patients is consistent with this hypothesis. The variability observed within the French GH cases could signify involvement of different prion strains, consistent with multiple contaminated GH batches in the French epidemic.

Conclusion The identification in this study of different PrPSc species in CJD patients with the same PRNP polymorphism at codon 129 and WB PrPres profile offers a new perspective on our understanding of the relationship between PrP biochemistry, prion disease phenotype and agent strain. We highlight two novel approaches to analysing PrPSc in sCJD and iCJD and offer evidence that these analyses provide potentially-strain associated information, which appears to be lacking from the conventional WB assay.



http://www.plospathogens.org/article/info:doi/10.1371/journal.ppat.1000029




Saturday, April 04, 2009 An unusually presenting case of sCJD—The VV1 subtype Volume 111, Issue 3, Pages 282-291 (April 2009)

An unusually presenting case of sCJD—The VV1 subtype

Kaloyan S. Taneva, Mimi Yilmab

Received 16 November 2007; received in revised form 4 September 2008; accepted 12 September 2008.

Abstract Creutzfeldt–Jakob disease (CJD) is a rapidly progressive neurodegenerative disease caused by prions. Typically CJD presents with a triad of rapidly progressive dementia, abnormal movements (e.g., myoclonus) and electroencephalographic (EEG) changes. Recently, CJD has been subdivided into subtypes based on host genetic polymorphisms and the characteristics of the pathological prion protein. Different subtypes likely have different clinical and laboratory presentations. We describe a case of sporadic CJD of the VV1 subtype. We describe our patient's clinical symptoms, time course, laboratory workup, structural and functional neuroimaging data, EEG data and CJD biomarkers. Our patient presented with clinical symptoms atypical for CJD. Because of that, her clinical symptoms were initially attributed to psychiatric reasons. After extensive clinical and laboratory investigation, we concluded that the patient probably had CJD. Postmortem neuropathological results confirmed this clinical hypothesis. We compare our patient's clinical, laboratory and neuroimaging data to the data on typical CJD as well as the data on the few CJD VV1 cases described in the literature. We discuss our case's relevance to the diagnosis of CJD.

Keywords: Creutzfeldt–Jakob disease, Dementia, Neuroimaging, Magnetic resonance imaging, Electroencephalography, Biomarkers, Prion diseases a Department of Psychiatry, Massachusetts General Hospital, 55 Fruit Street, Warren 1220/Blake 11, Boston, MA 02114, United States

b University of Connecticut Health Center, Farmington, CT, United States

Corresponding author. Tel.: +1 617 726 7511; fax: +1 617 724 9155.

PII: S0303-8467(08)00320-X

doi:10.1016/j.clineuro.2008.09.017

© 2008 Elsevier B.V. All rights reserved.

http://www.clineu-journal.com/article/S0303-8467(08)00320-X/abstract


rare atypical strain of sporadic cjd ??? seems these rare strains are increasing ???


Wednesday, February 04, 2009

Creutzfeldt-Jacob disease presenting as severe depression: a case report

http://creutzfeldt-jakob-disease.blogspot.com/2009/02/creutzfeldt-jacob-disease-presenting-as.html


A case-control study of sporadic Creutzfeldt-Jakob disease in Switzerland: analysis of potential risk factors with regard to an increased CJD incidence in the years 2001-2004

http://creutzfeldt-jakob-disease.blogspot.com/2009/02/case-control-study-of-sporadic.html


Thursday, July 10, 2008

A New Prionopathy update July 10, 2008

snip...

DOES ANYONE BESIDES ME SEE A PATTERN YET ???

Vickey Rimmer, 16, DID NOT DIE FROM nvCJD, she died from a form of sporadic CJD, whatever the hell that is. and there have been 16 year old die from sporadic CJD in the USA as well.

SIMPLY PUT, the ukbsenvcjd only theory was wrong from day one. the elderly are expendable, pets and kids are not.

Science was dictated by 'big buisness' after the Vickey Rimmer case with the ukbsenvcjd only myth.

snip...

Sporadic creutzfeldt-jakob disease in two adolescents

http://jnnp.bmj.com/cgi/content/abstract/jnnp.2006.104570v1


see full text sporadic CJD the big lie;

snip...

IT seems we have come full circle from the 'ORIGINAL 10' i.e. the 1st 10 adolescents in the UKBSEnvCJD only theory. and now we find us at the 1st 10 in USA, or is it the first 10, or the tip of the iceburg, many that went undocumented ???

lets look at the full circle, to date ;

http://cjdmadcowbaseoct2007.blogspot.com/2008/07/new-prionopathy-update-july-10-2008.html


Sunday, August 10, 2008

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



full text ;


http://creutzfeldt-jakob-disease.blogspot.com/2009/04/unusually-presenting-case-of-scjdthe.html




Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob disease in the United States 2003 revisited 2009

Saturday, July, 18, 2009

Greetings,

I would like to submit a review of past CJD surveillance in the USA, and the urgent need to make all human TSE in the USA a reportable disease, in every state, of every age group, and to make this mandatory immediately without further delay. The ramifications of not doing so will only allow this agent to spread further in the medical, dental, surgical arena’s. North America seems to have the most species with documented Transmissible Spongiform Encephalopathy's, most all of which have been rendered and fed back to food producing animals and to humans for years. If you look at the statistics, sporadic CJD seems to be rising in the USA, and has been, with atypical cases of the sCJD. I find deeply disturbing in the year of 2009, that Human Transmissible Spongiform Encephalopathy of any strain and or phenotype, of all age groups, and I stress all age groups, because human TSE's do not know age, and they do not know borders. someone 56 years old, that has a human TSE, that has surgery, can pass this TSE agent on i.e. friendly fire, and or passing it forward, and there have been documented nvCJD in a 74 year old. Remembering also that only sporadic CJD has been documented to transmit via iatrogenic routes, until recently with the 4 cases of blood related transmission, of which the origin is thought to be nvCJD donors. However most Iatrogenic CJD cases are nothing more than sporadic CJD, until the source is proven, then it becomes Iatrogenic. An oxymoron of sorts, because all sporadic CJD is, are multiple forms, or strains, or phenotypes of Creutzfeldt Jakob Disease, that the route and source and species have not been confirmed and or documented. When will the myth of the UKBSEnvCJD only theory be put to bed for good. This theory in my opinion, and the following there from, as the GOLD STANDARD, has done nothing more than help spread this agent around the globe. Politics and money have caused the terrible consequences to date, and the fact that TSEs are a slow incubating death, but a death that is 100% certain for those that are exposed and live long enough to go clinical. once clinical, there is no recourse, to date. But, while sub-clinical, how many can one exposed human infect? Can humans exposed to CWD and scrapie strains pass it forward as some form of sporadic CJD in the surgical and medical arenas? why must we wait decades and decades to prove this point, only to expose millions needlessly, only for the sake of the industries involved? would it not have been prudent from the beginning to just include all TSE's, and rule them out from there with transmission studies and change policies there from, as opposed to doing just the opposite? The science of TSE's have been nothing more than a political circus since the beginning, and for anyone to still believe in this one strain, one group of bovines, in one geographical location, with only one age group of human TSE i.e. nvCJD myth, for anyone to believe this today only enhances to spreading of these human and animal TSE's. This is exactly why we have been in this quagmire.

The ones that believe that there is a spontaneous CJD in 85%+ of all cases of human TSE, and the ones that do not believe that cattle can have this same phenomenon, are two of the same, the industry, and so goes the political science aspect of this tobacco and or asbestos scenario i.e. follow the money. I could go into all angles of this man made nightmare, the real facts and science, for instance, the continuing rendering technology and slow cooking with low temps that brewed this stew up, and the fact that THE USA HAD THIS TECHNOLOGY FIRST AND SHIPPED IT TO THE U.K. SOME 5 YEARS BEFORE THE U.S. STARTED USING THE SAME TECHNOLOGY, to save on fuel cost. This is what supposedly amplified the TSE agent via sheep scrapie, and spread via feed in the U.K. bovine, and other countries exporting the tainted product. BUT most everyone ignores this fact, and the fact that the U.S. has been recycling more TSE, from more species with TSEs, than any other country documented, but yet, it's all spontaneous, and the rise in sporadic CJD in the U.S. is a happenstance of bad luck ??? I respectfully disagree. To top that all off, the infamous BSE-FIREWALL that the USDA always brags about was nothing more than ink on paper, and I can prove this. YOU can ignore it, but this is FACT (see source, as late as 2007, in one recall alone, some 10,000,000 MILLION POUNDS OF BANNED MAD COW FEED WENT OUT INTO COMMERCE TO BE FED OUT, and most was never recovered. This was banned blood laced, meat and bone meal. 2006 was a banner year for banned mad cow protein going into commerce in the U.S. (see source of FDA feed ban warning letter below). I stress that the August 4, 1997 USA mad cow feed ban and this infamous BSE firewall, was nothing more than ink on paper, it was never enforceable.

I propose that the current diagnostic criteria for human TSEs only enhances and helps the spreading of human TSE from the continued belief of the UKBSEnvCJD only theory in 2009. With all the science to date refuting it, to continue to validate this old myth, will only spread this TSE agent through a multitude of potential routes and sources i.e. consumption, medical i.e., surgical, blood, dental, endoscopy, optical, nutritional supplements, cosmetics etc. I propose as with Aguzzi, Asante, Collinge, Caughey, Deslys, Dormont, Gibbs, Gajdusek, Ironside, Manuelidis, Marsh, et al and many more, that the world of TSE Transmissible Spongiform Encephalopathy is far from an exact science, but there is enough proven science to date that this myth should be put to rest once and for all, and that we move forward with a new classification for human and animal TSE that would properly identify the infected species, the source species, and then the route. This would further have to be broken down to strain of species and then the route of transmission would further have to be broken down. Accumulation and Transmission are key to the threshold from sub-clinical to clinical disease, and key to all this, is to stop the amplification and transmission of this agent, the spreading of, no matter what strain. In my opinion, to continue with this myth that the U.K. strain of BSE one strain TSE in cows, and the nv/v CJD one strain TSE humans, and the one geographical location source i.e. U.K., and that all the rest of human TSE are just one single strain i.e. sporadic CJD, a happenstance of bad luck that just happens due to a twisted protein that just twisted the wrong way, IN 85%+ OF ALL HUMAN TSEs, when to date there are 6 different phenotypes of sCJD, and growing per Gambetti et al, and that no other animal TSE transmits to humans ??? With all due respect to all Scientist that believe this, I beg to differ. To continue with this masquerade will only continue to spread, expose, and kill, who knows how many more in the years and decades to come. ONE was enough for me, My Mom, hvCJD i.e. Heidenhain Variant CJD, DOD 12/14/97 confirmed, which is nothing more than another mans name added to CJD, like CJD itself, Jakob and Creutzfeldt, or Gerstmann-Straussler-Scheinker syndrome, just another CJD or human TSE, named after another human. WE are only kidding ourselves with the current diagnostic criteria for human and animal TSE, especially differentiating between the nvCJD vs the sporadic CJD strains and then the GSS strains and also the FFI fatal familial insomnia strains or the ones that mimics one or the other of those TSE? Tissue infectivity and strain typing of the many variants 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. ...

please see history, and the ever evolving TSE science to date ;

Saturday, June 13, 2009

Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob disease in the United States 2003 revisited 2009

http://cjdusa.blogspot.com/2009/06/monitoring-occurrence-of-emerging-forms.html


SEE THE DAMNING VIDEO NOW AT THE BOTTOM OF THE BLOG BELOW ;

http://creutzfeldt-jakob-disease.blogspot.com/2009/07/usa-hiding-mad-cow-disease-victims-as.html



Saturday, August 01, 2009

Cases of Early-Onset Sporadic Creutzfeld-Jakob Disease in Michigan

http://creutzfeldt-jakob-disease.blogspot.com/2009/08/cases-of-early-onset-sporadic.html




2006


From: TSS
Subject: HUMAN and ANIMAL TSE Classifications i.e. mad cow disease and the UKBSEnvCJD only theory
Date: January 29, 2006 at 9:03 am PST
In Reply to: Tracking Spongiform Encephalopathies in North America (Lancet Infectious Disease Volume 3, Number 8 01 August 2003)
posted by TSS on August 14, 2003 at 6:56 pm:
------------------------------------------------------------
Comments sent via JAMA Feedback Page
------------------------------------------------------------
NAME: Terry S. Singeltary Sr.
E-MAIL: flounder9@verizon.net
PREVIOUS PAGE: http://jama.ama-assn.org/


Comments sent via JAMA Feedback Page

NAME: Terry S. Singeltary Sr. E-MAIL: flounder9@verizon.net




COMMENTS: I wish to submit the following ;

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

TSEs have been rampant in the USA for decades in many species, and they all have been rendered and fed back to animals for human/animal consumption. I propose that the current diagnostic criteria for human TSEs only enhances and helps the spreading of human TSE from the continued belief of the UKBSEnvCJD only theory in 2005. With all the science to date refuting it, to continue to validate this myth, will only spread this TSE agent through a multitude of potential routes and sources...snip...end...TSS



Monday, April 20, 2009

National Prion Disease Pathology Surveillance Center Cases Examined1 (December 31, 2008)

http://prionunitusaupdate2008.blogspot.com/2009/04/national-prion-disease-pathology.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 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







Tuesday, July 14, 2009

U.S. Emergency Bovine Spongiform Encephalopathy Response Plan Summary and BSE Red Book Date: February 14, 2000 at 8:56 am PST

WHERE did we go wrong $$$

http://madcowtesting.blogspot.com/2009/07/us-emergency-bovine-spongiform.html


Transgenic mice expressing porcine prion protein resistant to classical scrapie but susceptible to sheep bovine spongiform encephalopathy and atypical scrapie. Emerg Infect Dis. 2009 Aug; [Epub ahead of print]

http://nor-98.blogspot.com/2009/07/transgenic-mice-expressing-porcine.html


Transmissible mink encephalopathy - review of the etiology

http://transmissible-mink-encephalopathy.blogspot.com/2009/07/transmissible-mink-encephalopathy.html


Wednesday, July 1, 2009

Nor98 scrapie identified in the United States J Vet Diagn Invest 21:454-463 (2009)

http://nor-98.blogspot.com/2009/07/nor98-scrapie-identified-in-united.html


Monday, June 01, 2009 Biochemical typing of pathological prion protein in aging cattle with BSE

SOMETHING TO PONDER ???

O.K. confusious asks, IF all these new atypical BSEs i.e. new strains of mad cow disease is just an 'OLD COW PRION DISEASE', why then can not the 'old human prion disease' such as the sporadic CJD, be from an 'old cow prion disease', same as the nvCJD 'young people mad cow disease' (which also happens in 74 year old), but why cannot the 'old cow prion diseases', i.e. l-BSE, h-BSE, and ibncBSE, cause the 'old people prion disease', which looks like sporadic CJD. seems that is what some of the pathology is showing ???

OH, that probably makes too much sense, and that the only answer could be that it's all just a happenstance of bad luck and or a spontaneous event, that just happens out of the clear blue sky $$$

IF this is the case, then where are all the SPONTANEOUS BSE CASES OF MAD COW DISEASE IN THE U.S.A., AND WHERE HAVE THEY BEEN BURIED IN THE USA OVER THE LAST 25 YEARS ???

http://bse-atypical.blogspot.com/2009/06/biochemical-typing-of-pathological.html


Tuesday, August 04, 2009

Susceptibilities of Nonhuman Primates to Chronic Wasting Disease

http://chronic-wasting-disease.blogspot.com/2009/08/susceptibilities-of-nonhuman-primates.html


Thursday, July 23, 2009

UW Hospital warning 53 patients about possible exposure to rare brain disease

http://creutzfeldt-jakob-disease.blogspot.com/2009/07/uw-hospital-warning-53-patients-about.html


Wednesday, August 05, 2009 Rate of CWD infection increases in core area WISCONSIN

http://chronic-wasting-disease.blogspot.com/2009/08/rate-of-cwd-infection-increases-in-core.html


Terry S. Singeltary Sr. P.O. Box 42 Bacliff, Texas USA 77518