CJD FOUNDATION CWRU GAMBETTI FAMILIAL FAMILY AFFAIR CONFERENCE 2012
CJD FOUNDATION CWRU GAMBETTI FAMILIAL FAMILY AFFAIR CONFERENCE 2012
Greetings CJDVoice, bloodcjd et al,
LOOKS like another familial family affair with the CJD Foundation CWRU Gambetti Conference for 2012.
SOMEWHERE along the lines, the sporadic CJD victims and families were hung out to dry $$$
just my ongoing opinion. ...TSS
TENTATIVE-3/20/12
CJD 2012 and the Tenth Annual CJD Foundation Family Conference
Friday, July 13th
11:00 – 3:30 Registration
10:00 – 11:00 CJDISA Friends & Advisors meeting
*This meeting is open only to the members of the CJDISA Friends & Advisors Committee
11:30 – 12:45 CJD Foundation Board of Directors Meeting
*This meeting is open only to the members of the CJD Foundation Board of Directors
1:00 – 3:00 Familial Prion Disease Meeting
*This meeting is only for families affected by a familial prion disease
Deana Simpson, RN – Chairman
Founder and Director, CJD Insight; Member, CJD Foundation Board of Directors
Macomb, Michigan
Pierluigi Gambetti, MD
Professor, Case Western Reserve University School of Medicine,
Department of Pathology
Director, The National Prion Disease Pathology Surveillance Center;
Medical Director, The CJD Foundation, CJD Foundation Board of Directors
Cleveland, Ohio
Michael Geschwind, MD, PhD
Assistant Adjunct Professor of Neurology, Memory and Aging Center, University of
California at San Francisco
San Francisco, California
Eva Mitrova´, M.D.
National Reference Centre for Prion Diseases,
Research base of Slovak Medical University,
Limbova´ str. 14, 833 03
Bratislava, Slovakia
2
3:15 - 5:15 Bereavement Workshop
Deana Simpson, RN, Co-chairman
Katrina Hallmark, PsyD, Co-chairman
Neuropsychologist, Southwest Medical Center
San Antonio, Texas
Brian Appleby, MD
Lou Ruvo Center for Brain Health, Neurological Institute
Cleveland Clinic Foundation
Cleveland, Ohio
4:30 – 7:30 Registration
6:00 – 7:30 Welcome Reception
Saturday, July 14th
7:00 a.m. Conference Registration
7:00 – 8:00 Continental Breakfast
8:00 – 8:15 Welcome
Florence Kranitz
President, The CJD Foundation; Co-Chairman, The CJD International Support Alliance
Akron, Ohio
8:15 – 8:40 Memorial Service
Co-Chairmen: Pierluigi Gambetti, MD
Florence Kranitz
Keynote Address
8:40 - 9:10 Byron Caughey, PhD
New Ways of Prion Disease Detection and Diagnosis
Senior Investigator
Chief, TSE/prion Biochemistry Section
Laboratory of Persistent Viral Diseases
NIH/NIAID Rocky Mountain Laboratories
Hamilton, Montana
9:10 – 9:15 Q&A
3
New Discoveries
9:15 – 9:35 Emiliano Biasini, PhD
Novel Anti-Prion Compounds Identified by Targeting the Functional Activity of PrPC
Instructor, Harris Laboratory, Boston University
Boston, Massachusetts
Recipient of the Carlton Madderly Wilson and CJD Foundation Research Grant, 2011
9:35 - 9:40 Q&A
9:40 – 10:00 Edward Málaga-Trillo, Ph.D.
The Use of Zebra Fish to Study PrP Function and the Molecular Basis of Neurodegeneration
University of Konstanz, Department of Biology, Prion Biology Group
Konstanz, Germany
10:00 – 10:05 Q&A
10:05 – 10:15 Coffee Break
10:15 - 10:35 Neil Cashman, MD, FRCP(C), FCAHS
Understanding CJD Leads to New Treatments for ALS
Professor & Scientific Director, PrioNet Canada, a Network of Centres of Excellence
Vancouver, British Columbia
10:35 – 10:40 Q&A
10:40 - 11:00 Glenn Telling, PhD
The Spread of Chronic Wasting Disease and the Alteration of the Species Barrier by In Vitro Amplification
Director and Professor, Prion Research Center
Department of Microbiology, Immunology & Pathology Colorado State University
Fort Collins, Colorado
11:00 - 11:05 Q&A
11:05 - 11:25 Eva Mitrova´, M.D.
The Role of Anticipation in the Incidence of CJD with the Mutation E200K
11:25 - 11:30 Q&A
11:30 - 12:45 Lunch
4
Surveillance
12:45 - 1:05 Robert G. Will, MD, FRCP(E)
European Surveillance Overview
Professor of Neurology, Personal Chair in Clinical Neurology, University of Edinburgh;
Consultant Neurologist, Western General Hospital; Former Director and Founder,
National CJD Surveillance Unit
Edinburgh, United Kingdom
1:05 - 1:10 Q&A
1:10 - 1:30 Pierluigi Gambetti, MD
U.S. Prion Disease Surveillance and the Importance of Autopsy; Tests Conducted
1:30 - 1:35 Q&A
1:35 – 1:55 Daniel Krewski, MHA, MSc, PhD
Summary
Professor and Director of the R. Samuel McLaughlin Centre for Population Health Risk Assessment, University of Ottawa
Ottawa, Ontario
1:55 - 2:00 Q&A
2:00 - 2:10 Coffee Break
2:10 - 4:15 Moving Round Tables
7:00 – 7:30 Reception
7:30 Banquet
Sunday, July 15th
Co-Chairmen: Ruthie George Lori Nusbaum
7:30 – 8:45 Continental Breakfast
9:00 – 9:15 Mark Goldfarb
CJDF Update
Chairman, Board of Directors, The CJD Foundation
Akron, Ohio
5
Ruthie George
Treasurer, Board of Directors, The CJD Foundation
Akron, Ohio
9:15 - 9:30 Lori Nusbaum
Questionnaire Report
Associate Director, The CJD Foundation
Akron, Ohio
9:30 - 9:35 Q&A
9:35 – 10:05 Bradley Kalinsky, MD & Amanda Baxley Kalinsky, RN, BSN
In Vitro Options, A Personal Story
Nashville, Tennessee
10:05 – 10:10 Q&A
10:10 – 11:20 Panel Presentations
Joe Abrams, MPH
Centers for Disease Control and Prevention
NCEZID/DHCPP
Atlanta, Georgia
David Asher, MD
US Food and Drug Administration
Chief and Supervisory Medical Officer in the Laboratory of Bacterial and Transmissible
Spongiform Encephalopathy Agents within FDA’s Division of Emerging and
Transfusion-Transmitted Diseases, Office of Blood Research and Review,
Center for Biologics Evaluation and Research (CBER).
Rockville, Maryland
Marie Kassai RN, BSN, MPH, CIC
On behalf of World Health Organization
Infection Preventionist
Passaic, New Jersey
Christopher A. Waldrop, MPH
Consumer Federation of America
Director, Food Policy Institute
Washington, DC
11:20 - 11:30 Q&A
11:30 - 12:45 Lunch
6
Support Organization Reports
12:45 – 2:15 CJD International Support Alliance Panel Presentation
Introduction, Florence Kranitz & Suzanne Solvyns
Co Chairmen, The CJD International Support Network
Suzanne Solvyns
CJD Support Group Network, Australia
Director, CJD Support Group Network,
Co chairman the CJD International Support Group Network
Sydney NSW, Australia
David Ralston
CJD Support Group Network, Australia
Chair, CJD Support Group Network
Sydney NSW, Australia
Roberto Borgis
Associazione Italiana Encefalopatie da Prioni (A.I.En.P.), Italy
President, A.I.En.P.
Torino, Italy
J.B. Matthieu
MCJ-HCC, France
President, MCJ-HCC
Paris, France
Professor Richard Knight, MD, FRCP(E)
CJD Support Network, U.K.
Medical Director, CJD Support Network, U.K.
Edinburgh, Scotland
Victor Sanchez, MD, PhD
The CJD Foundation, Mexico
Director
Founder and Director, Mexican Survellance
Guadalajara, Mexcio
2:15 – 2:30 Coffee Break
2:30 – 4:15 Advocacy Training
Monday, July 16th
Capitol Hill Visits
http://www.cjdfoundation.org/pdfs/2012conferenceschedule.pdf
also, be warned, these folks don’t like questions being asked that are not screened.
“At the CJD Foundation conference in 2000 our members were accused of 'attacking' the researchers in the question and answer session because of the questions we were asking”.
speaking about that CJD Questionnaire the CJD Foundation and CWRU Gambetti et al are so proud of.
some folks here might want to review some old history about that, and others here might wish I would forget.
not going to happen. ...
Subject: Re: Tracie CJD Foundation
Date: Tue, 5 Nov 2002 15:09:29 -0500
From: "Tracie Kedzierski"
To: "Terry S. Singeltary Sr."
References: <3DC730DF.8010308@wt.net><018601c284f7$27a04a80$0a64a8c0@newportrentalguide .com><3DC80F1A.3090808@wt.net><000901c28502$fac15c00$0a64a8c0@newportrentalguide .com><3DC81FCA.1040403@wt.net>
Terry,
Oh no....I've gone and pissed you off (ha ha)I just find it better to speak...so that nothing I write is misinterpreted. It is very important to me that you understand the conflict, the confusion, etc so can I call you or not? My dime ?
Tracie
----- Original Message -----
From: "Terry S. Singeltary Sr."
To: "Tracie Kedzierski"
Sent: Tuesday, November 05, 2002 2:45 PM
Subject: Re: Tracie CJD Foundation
> either mail me your explination or forget the it...TSS
>> Tracie Kedzierski wrote:
>> > Terry,
The only problem is that having it on our message board conflicts with the information I have on our home page about the surveillance project and the report form I send out to the families. -----it is confusing. In fact.. I'm sorry but we (The Foundation) have to pull it off. I need to talk to you about this and share a number of goals the "new" Foundation has Can I call you? Please email me your number.... Tracie
> > Original Message -----
> > From: "Terry S. Singeltary Sr." > >
To: "Tracie Kedzierski"
Sent: Tuesday, November 05, 2002 1:34 PM
Subject: Re: Tracie CJD Foundation
hi Tracie,
doing fine, thank you. about the questionnaire? by no means am i trying to step on Dr Gambetti's toes here. i think there is more to it than just reporting a case. we _must_ find the source and route of spordic CJDs, and i think a great deal of it will be from the medical/surgical arena. i just want a questionnaire made up for _all_ victims of human TSEs in the USA in _every_ state, and i want it reportable in _every_ state. i am turning the heat up. ****, i'm getting old and grey, i want to see it done before i die. will be sending this out to many media and papers and requesting them to turn the heat up on the Gov. you will be able to keep up with the ones coming through the voice and if i get some that have not come through the list, i will pass on to Dr. Gambetti if he likes. i respect Dr. Gambetti very much and would do nothing to hender his work or yours. i just think that this is too important of a matter not to have one. and i think by turning the heat up, getting to the media and pressing there buttons a bit, just might help this get done a bit faster... hope so anyway...
kindest regards,
terry
>>Tracie Kedzierski wrote:> >>> >>>
Hi Terry,
How are you? I'm just curious about your Questionnaire ?
It just was posted on the Foundation's Message Board without any introduction... and I was a bit concerned as it may cause some confusion with the Surveillance Project I'm doing via the Foundation for Dr Gambetti. Could you let me know?
Tracie
Greetings again Voice,
just what is the _new_ CJD Foundations goals witha CJD Questionnaire that asks _no_
questions about soure/route of the six variants of sporadic CJDs???
=================================================
i am reminded of a few things deep throat told me years ago;
=================================================
The most frightening thing I have read all day is the report of Gambetti's finding of a new strain of sporadic cjd in young people......... Dear God, what in the name of all that is holy is that!!! If the US has different strains of scrapie..... why???? than the UK... then would the same mechanisms that make different strains of scrapie here make different strains of BSE... if the patterns are different in sheep and mice for scrapie..... could not the BSE be different in the cattle, in the mink, in the humans....... I really think the slides or tissues and everything from these young people with the new strain of sporadic cjd should be put up to be analyzed by many, many experts in cjd........ bse..... scrapie
Scrape the damn slide and put it into mice..... wait..... chop up the mouse brain and and spinal cord........ put into some more mice..... dammit amplify the thing and start the damned research..... This is NOT rocket science... we need to use what we know and get off our butts and move.... the whining about how long everything takes..... well it takes a whole lot longer if you whine for a year and then start the research!!!
Not sure where I read this but it was a recent press release or something like that: I thought I would fall out of my chair when I read about how there was no worry about infectivity from a histopath slide or tissues because they are preserved in formic acid, or formalin or formaldehyde..... for God's sake........ Ask any pathologist in the UK what the brain tissues in the formalin looks like after a year....... it is a big fat sponge... the agent continues to eat the brain ...... you can't make slides anymore because the agent has never stopped........ and the old slides that are stained with Hemolysin and Eosin...... they get holier and holier and degenerate and continue... what you looked at 6 months ago is not there........ Gambetti better be photographing every damned thing he is looking at.....
Okay, you need to know. You don't need to pass it on as nothing will come of it and there is not a damned thing anyone can do about it. Don't even hint at it as it will be denied and laughed at.......... USDA is gonna do as little as possible until there is actually a human case in the USA of the nvcjd........ if you want to move this thing along and shake the earth.... then we gotta get the victims families to make sure whoever is doing the autopsy is credible, trustworthy, and a saint with the courage of Joan of Arc........ I am not kidding!!!! so, unless we get a human death from EXACTLY the same form with EXACTLY the same histopath lesions as seen in the UK nvcjd........ forget any action........ it is ALL gonna be sporadic!!! And, if there is a case....... there is gonna be every effort to link it to international travel, international food, etc. etc. etc. etc. etc. They will go so far as to find out if a sex partner had ever traveled to the UK/europe, etc. etc. .... It is gonna be a long, lonely, dangerous twisted journey to the truth. They have all the cards, all the money, and are willing to threaten and carry out those threats.... and this may be their biggest downfall...
Thanks as always for your help. (Recently had a very startling revelation from a rather senior person in government here.......... knocked me out of my chair........ you must keep pushing. If I was a power person.... I would be demanding that there be at least a million bovine tested as soon as possible and agressively seeking this disease. The big players are coming out of the wood work as there is money to be made!!!
In short: "FIRE AT WILL"!!! for the very dumb.... who's "will"! "Will be the burden to bare if there is any coverup!"
again it was said years ago and it should be taken seriously.... BSE will NEVER be found in the US!
As for the BSE conference call... I think you did agreat service to freedom of information and making some people feign integrity... I find it scary to see that most of the "experts" are employed by the federal government or are supported on the "teat" of federal funds. A scary picture! I hope there is a confidential panel organized by the new government to really investigate this thing.
You need to watch your back........ but keep picking at them....... like a buzzard to the bone... you just may get to the truth!!! (You probably have more support than you know. Too many people are afraid to show you or let anyone else know. I have heard a few things myself... you ask the questions that everyone else is too afraid to ask.)
================================================
greetings again voice,
then i remind everyone to read this;
'As implied in the Inset 25 we must not assume that transmission of BSE to other species will invariably present pathology typical of a scrapie-like disease.'
http://www.bseinquiry.gov.uk/files/yb/1991/01/04004001.pdf
TSS
#################### https://lists.aegee.org/bse-l.html ####################
FOR THOSE THAT MIGHT BE INTERESTED, see the CJD Foundation CWRU prion unit old original CJD Questionnaire, compared to the one I proposed here (others on cjdvoice helped add questions). the CJD Foundation and cwru et al were very upset about the CJD Questionnaire and the questions pertaining to _all_ potential routes and sources of the TSE prion disease I put on the web, and some were filling out and sending to me. the CJD questionnaire at the time the CJD foundation cwru Gambetti prion unit had, ask NO questions pertaining to route and source. all it ask was how CJD was diagnosed, and by whom, where, etc. NOTHING about route and source. we hounded them and hounded them about this. once they proposed one that did ask a few questions, years later, they then did not give it out to everyone. they picked and chose who got one. now, today, I don’t know how the CJD Questionnaire is handled. they were so proud of what WE did (they forgot about that, or refused to acknowledge that), they then went on to congratulate everyone that _opposed_ the CJD Questionnaire at first, the ones that refused to ask any questions pertaining to potential routes and sources, at one of the CJD Foundations conferences. nope, they did not like that at all. but here is the history on that. some may not like this, but I will bring it up every year I have a breath of air left in me. Cele was right all along. ...
Friday, November 30, 2007
CJD QUESTIONNAIRE USA CWRU AND CJD FOUNDATION
http://cjdquestionnaire.blogspot.com/2007/11/cjd-questionnaire.html
http://cjdquestionnaire.blogspot.com/
Subject: Re: CJD FOUNDATION CONFERENCE A STEP BACKWARD, bending over to be non-controversial (amen)
Date: Mon, 20 Jan 2003 12:17:10 –0600
From: "Terry S. Singeltary Sr."
To: John Stauber
CC: bloodcjd
References:
hi john,
John Stauber wrote:
> --- drew my attention to the agenda for the upcoming CJD
> Foundation conference. Beth Willaims is doing a presentation
> on 'does CWD cause CJD'. The conference agenda seems a big
> step backwards, bending over to be non-controversial. This is
> typical with what happens to "disease victim" organizations,
> unforunately.
my friend, i could not agree more. just another...SNIP...END...TSS
kind regards,
terry
VARIABLY PROTEASE-SENSITVE PRIONOPATHY IS TRANSMISSIBLE ...price of prion poker goes up again $
OR-10 15:25 - 15:40 VARIABLY PROTEASE-SENSITIVE PRIONOPATHY IS TRANSMISSIBLE IN BANK VOLES Nonno
http://www.prion2012.com/program/final-program
I believe it was Gambetti et al that coined this term sporadic FFI, from some conspicuous sub-type of sporadic CJD possibly? seems they could not tie it to a true FFI by diagnostic standards to date, so it was then termed a sFFI, confusing matters even worse. ...
A subtype of sporadic prion disease mimicking fatal familial insomnia
http://www.neurology.org/cgi/content/abstract/52/9/1757?ck=nck
THIS seems to raise more questions than answers, confusing the TSEs even worse.
WHAT is sporadic CJD, and how many sub-types and atypical strains, phenotypes etc. will there be, arising from nothing. a spontaneous happening of sorts???
i think not. ...tss
http://sporadicffi.blogspot.com/
Wednesday, October 27, 2010
A novel variant of human disease with a protease-sensitive prion protein and heterozygosity methionine/valine at codon 129: Case report
snip...
Genetic findings
No mutations were found in the open reading frame after sequencing the prion protein gene (PRNP). A heterozygosis methionine valine (MV) was observed in codon 129.
snip...
http://creutzfeldt-jakob-disease.blogspot.com/2010/10/novel-variant-of-human-disease-with.html
Thursday, July 10, 2008
A Novel Human Disease with Abnormal Prion Protein Sensitive to Protease update July 10, 2008
Although several subjects had family histories of dementia, no mutations were found in the PrP gene open reading frame.
http://cjdmadcowbaseoct2007.blogspot.com/2008/07/novel-human-disease-with-abnormal-prion.html
Thursday, July 10, 2008
A New Prionopathy update July 10, 2008
http://cjdmadcowbaseoct2007.blogspot.com/2008/07/new-prionopathy-update-july-10-2008.html
***+++***
Thursday, July 10, 2008
A Novel Human Disease with Abnormal Prion Protein Sensitive to Protease update July 10, 2008
Friday, June 20, 2008
http://cjdmadcowbaseoct2007.blogspot.com/2008/07/novel-human-disease-with-abnormal-prion.html
Here we go folks. AS predicted. THIS JUST OUT !
Tuesday, August 03, 2010
Variably protease-sensitive prionopathy: A new sporadic disease of the prion protein
http://creutzfeldt-jakob-disease.blogspot.com/2010/08/variably-protease-sensitive-prionopathy.html
Monday, August 9, 2010
Variably protease-sensitive prionopathy: A new sporadic disease of the prion protein or just more PRIONBALONEY ?
snip...see full text ;
http://transmissiblespongiformencephalopathy.blogspot.com/2011/05/variably-protease-sensitive-prionopathy.html
http://prionopathy.blogspot.com/2011/05/variably-protease-sensitive-prionopathy.html
O.K. let's compare some recent cases of this prionpathy in other countries besides Gambetti's first 10 recently, that he claims is a spontaneous event, from a genetic disorder, that is not genetic, but sporadic, that is related to no animal TSE in North America, or the world. ...
http://prionunitusaupdate2008.blogspot.com/2010/08/variably-protease-sensitive-prionopathy.html
Wednesday, October 27, 2010
A novel variant of human disease with a protease-sensitive prion protein and heterozygosity methionine/valine at codon 129: Case report
http://creutzfeldt-jakob-disease.blogspot.com/2010/10/novel-variant-of-human-disease-with.html
Sunday, August 09, 2009
CJD...Straight talk with...James Ironside...and...Terry Singeltary... 2009
http://creutzfeldt-jakob-disease.blogspot.com/2009/08/cjdstraight-talk-withjames.html
Tuesday, August 18, 2009
BSE-The Untold Story - joe gibbs and singeltary 1999 – 2009
http://madcowusda.blogspot.com/2009/08/bse-untold-story-joe-gibbs-and.html
====================================
The familial mutations, Gajdusek proposed, lowered the barrier to such accidental conversion. "Thus," he wrote in 1996, "with these mutations, this ordinarily rare event becomes a ... dominant inherited trait." But Weissmann's qualification still remained to be refuted: the mutations might simply allow easier entry to a lurking virus. ...page 202 Deadly Feast
===================================
something to think about for sure.
but i interpret this as (1st not the gold standard, just my opinion;-), as because of certain gene mutations, one or a family, would be more susceptible to the many different strains of TSE, and the many different proven routes and sources, (which will cause different symptoms, different incubation periods from onset of clinical symptoms to death, different parts of the brain infected, etc.). in other words, it's NOT the gene mutation that CAUSES the disease, but the fact that it makes you more SUSCEPTIBLE, to the TSEs from the surrounding environment, and PLUS accumulation, i think this plays a critical role. maybe there is a one dose scenario, but i think there is more of the 'accumulators' that go clinical, than the 'one dose'. and what is the threshold to sub-clinical to clinical ?
anyway, just pondering out loud here.
also, for anyone interested, there are some studies with links to follow here ;
http://sporadicffi.blogspot.com/
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
LET'S take a closer look at this new prionpathy or prionopathy, and then let's look at the g-h-BSEalabama mad cow. This new prionopathy in humans? the genetic makeup is IDENTICAL to the g-h-BSEalabama mad cow, the only _documented_ mad cow in the world to date like this, ......wait, it get's better. this new prionpathy is killing young and old humans, with LONG DURATION from onset of symptoms to death, and the symptoms are very similar to nvCJD victims, OH, and the plaques are very similar in some cases too, bbbut, it's not related to the g-h-BSEalabama cow, WAIT NOW, it gets even better, the new human prionpathy that they claim is a genetic TSE, has no relation to any gene mutation in that family. daaa, ya think it could be related to that mad cow with the same genetic make-up ??? there were literally tons and tons of banned mad cow protein in Alabama in commerce, and none of it transmitted to cows, and the cows to humans there from ??? r i g h t $$$ ALABAMA MAD COW g-h-BSEalabama In this study, we identified a novel mutation in the bovine prion protein gene (Prnp), called E211K, of a confirmed BSE positive cow from Alabama, United States of America. This mutation is identical to the E200K pathogenic mutation found in humans with a genetic form of CJD. This finding represents the first report of a confirmed case of BSE with a potential pathogenic mutation within the bovine Prnp gene. We hypothesize that the bovine Prnp E211K mutation most likely has caused BSE in "the approximately 10-year-old cow" carrying the E221K mutation.
http://www.plospathogens.org/article/info%3Adoi%2F10.1371%2Fjournal.ppat.1000156
http://www.plospathogens.org/article/fetchObjectAttachment.action?uri=info%3Adoi%2F10.1371%2Fjournal.ppat.1000156&representation=PDF
Saturday, August 14, 2010
BSE Case Associated with Prion Protein Gene Mutation (g-h-BSEalabama) and VPSPr PRIONPATHY (see mad cow feed in COMMERCE IN ALABAMA...TSS)
http://prionpathy.blogspot.com/2010/08/bse-case-associated-with-prion-protein.html
her healthy calf also carried the mutation (J. A. Richt and S. M. Hall PLoS Pathog. 4, e1000156; 2008).
This raises the possibility that the disease could occasionally be genetic in origin. Indeed, the report of the UK BSE Inquiry in 2000 suggested that the UK epidemic had most likely originated from such a mutation and argued against the scrapierelated assumption. Such rare potential pathogenic PRNP mutations could occur in countries at present considered to be free of BSE, such as Australia and New Zealand. So it is important to maintain strict surveillance for BSE in cattle, with rigorous enforcement of the ruminant feed ban (many countries still feed ruminant proteins to pigs). Removal of specified risk material, such as brain and spinal cord, from cattle at slaughter prevents infected material from entering the human food chain. Routine genetic screening of cattle for PRNP mutations, which is now available, could provide additional data on the risk to the public. Because the point mutation identified in the Alabama animals is identical to that responsible for the commonest type of familial (genetic) CJD in humans, it is possible that the resulting infective prion protein might cross the bovine-human species barrier more easily. Patients with vCJD continue to be identified. The fact that this is happening less often should not lead to relaxation of the controls necessary to prevent future outbreaks. Malcolm A. Ferguson-Smith Cambridge University Department of Veterinary Medicine, Madingley Road, Cambridge CB3 0ES, UK e-mail: mailto:maf12%40cam.ac.uk Jürgen A. Richt College of Veterinary Medicine, Kansas State University, K224B Mosier Hall, Manhattan, Kansas 66506-5601, USA NATURE|Vol 457|26 February 2009
http://www.nature.com/nature/journal/v457/n7233/full/4571079b.html
SEE FULL TEXT OF ALL THIS HERE ;
2009 UPDATE ON ALABAMA AND TEXAS MAD COWS 2005 and 2006
http://bse-atypical.blogspot.com/2006/08/bse-atypical-texas-and-alabama-update.html
Saturday, June 25, 2011
Transmissibility of BSE-L and Cattle-Adapted TME Prion Strain to Cynomolgus Macaque
"BSE-L in North America may have existed for decades"
http://transmissiblespongiformencephalopathy.blogspot.com/2011/06/transmissibility-of-bse-l-and-cattle.html
Over the next 8-10 weeks, approximately 40% of all the adult mink on the farm died from TME.
snip...
The rancher was a ''dead stock'' feeder using mostly (>95%) downer or dead dairy cattle...
http://web.archive.org/web/20030516051623/http://www.bseinquiry.gov.uk/files/mb/m09/tab05.pdf
Sunday, June 26, 2011
Risk Analysis of Low-Dose Prion Exposures in Cynomolgus Macaque
http://transmissiblespongiformencephalopathy.blogspot.com/2011/06/risk-analysis-of-low-dose-prion.html
Monday, September 26, 2011
L-BSE BASE prion and atypical sporadic CJD
http://bse-atypical.blogspot.com/2011/09/l-bse-base-prion-and-atypical-sporadic.html
for more, see full text here ;
Monday, September 26, 2011
Variably Protease-Sensitive Prionopathy, Prionpathy, Prionopathy, FFI, GSS, gCJD, hvCJD, sCJD, TSE, PRION, update 2011
http://prionopathy.blogspot.com/2011/09/variably-protease-sensitive-prionopathy.html
DEEP THROAT TO TSS 2000-2001 (take these old snips of emails with how ever many grains of salt you wish. ...tss)
The most frightening thing I have read all day is the report of Gambetti's finding of a new strain of sporadic cjd in young people...Dear God, what in the name of all that is holy is that!!! If the US has different strains of scrapie.....why????than the UK...then would the same mechanisms that make different strains of scrapie here make different strains of BSE...if the patterns are different in sheep and mice for scrapie.....could not the BSE be different in the cattle, in the mink, in the humans.......I really think the slides or tissues and everything from these young people with the new strain of sporadic cjd should be put up to be analyzed by many, many experts in cjd........bse.....scrapie Scrape the damn slide and put it into mice.....wait.....chop up the mouse brain and and spinal cord........put into some more mice.....dammit amplify the thing and start the damned research.....This is NOT rocket science...we need to use what we know and get off our butts and move....the whining about how long everything takes.....well it takes a whole lot longer if you whine for a year and then start the research!!! Not sure where I read this but it was a recent press release or something like that: I thought I would fall out of my chair when I read about how there was no worry about infectivity from a histopath slide or tissues because they are preserved in formic acid, or formalin or formaldehyde.....for God's sake........ Ask any pathologist in the UK what the brain tissues in the formalin looks like after a year.......it is a big fat sponge...the agent continues to eat the brain ......you can't make slides anymore because the agent has never stopped........and the old slides that are stained with Hemolysin and Eosin......they get holier and holier and degenerate and continue...what you looked at 6 months ago is not there........Gambetti better be photographing every damned thing he is looking at.....
Okay, you need to know. You don't need to pass it on as nothing will come of it and there is not a damned thing anyone can do about it. Don't even hint at it as it will be denied and laughed at.......... USDA is gonna do as little as possible until there is actually a human case in the USA of the nvcjd........if you want to move this thing along and shake the earth....then we gotta get the victims families to make sure whoever is doing the autopsy is credible, trustworthy, and a saint with the courage of Joan of Arc........I am not kidding!!!! so, unless we get a human death from EXACTLY the same form with EXACTLY the same histopath lesions as seen in the UK nvcjd........forget any action........it is ALL gonna be sporadic!!!
And, if there is a case.......there is gonna be every effort to link it to international travel, international food, etc. etc. etc. etc. etc. They will go so far as to find out if a sex partner had ever traveled to the UK/europe, etc. etc. .... It is gonna be a long, lonely, dangerous twisted journey to the truth. They have all the cards, all the money, and are willing to threaten and carry out those threats....and this may be their biggest downfall...
Thanks as always for your help. (Recently had a very startling revelation from a rather senior person in government here..........knocked me out of my chair........you must keep pushing. If I was a power person....I would be demanding that there be a least a million bovine tested as soon as possible and agressively seeking this disease. The big players are coming out of the woodwork as there is money to be made!!! In short: "FIRE AT WILL"!!! for the very dumb....who's "will"! "Will be the burden to bare if there is any coverup!"
again it was said years ago and it should be taken seriously....BSE will NEVER be found in the US! As for the BSE conference call...I think you did a great service to freedom of information and making some people feign integrity...I find it scary to see that most of the "experts" are employed by the federal government or are supported on the "teat" of federal funds. A scary picture! I hope there is a confidential panel organized by the new government to really investigate this thing.
You need to watch your back........but keep picking at them.......like a buzzard to the bone...you just may get to the truth!!! (You probably have more support than you know. Too many people are afraid to show you or let anyone else know. I have heard a few things myself... you ask the questions that everyone else is too afraid to ask.)
END...TSS
IN CONFIDENCE
Perceptions of unconventional slow virus in the USA
GAH WELLS
Report of a visit to the U.S.A. April-May 1989
3. Prof. A Robertson gave a brief account of BSE. The US approach was to accord it a very low profile indeed.
Dr. A Thiermann showed the picture in the ''Independent'' with cattle being incinerated and thought this was a fantical incident to be avoided in the USA AT ALL COSTS.
http://web.archive.org/web/20060307063531/http://www.bseinquiry.gov.uk/files/mb/m11b/tab01.pdf
and they meant it !
Monday, March 19, 2012
Infectivity in Skeletal Muscle of Cattle with Atypical Bovine Spongiform Encephalopathy
PLoS One. 2012; 7(2): e31449.
http://transmissiblespongiformencephalopathy.blogspot.com/2012/03/infectivity-in-skeletal-muscle-of.html
Saturday, March 5, 2011
MAD COW ATYPICAL CJD PRION TSE CASES WITH CLASSIFICATIONS PENDING ON THE RISE IN NORTH AMERICA
http://transmissiblespongiformencephalopathy.blogspot.com/2011/03/mad-cow-atypical-cjd-prion-tse-cases.html
Sunday, February 12, 2012
National Prion Disease Pathology Surveillance Center Cases Examined1 (August 19, 2011) including Texas
http://transmissiblespongiformencephalopathy.blogspot.com/2012/02/national-prion-disease-pathology.html
kind regards, terry
Wednesday, March 28, 2012
VARIABLY PROTEASE-SENSITVE PRIONOPATHY IS TRANSMISSIBLE, price of prion poker goes up again $
http://prionopathy.blogspot.com/2012/03/variably-protease-sensitve-prionopathy.html
TSS
Greetings CJDVoice, bloodcjd et al,
LOOKS like another familial family affair with the CJD Foundation CWRU Gambetti Conference for 2012.
SOMEWHERE along the lines, the sporadic CJD victims and families were hung out to dry $$$
just my ongoing opinion. ...TSS
TENTATIVE-3/20/12
CJD 2012 and the Tenth Annual CJD Foundation Family Conference
Friday, July 13th
11:00 – 3:30 Registration
10:00 – 11:00 CJDISA Friends & Advisors meeting
*This meeting is open only to the members of the CJDISA Friends & Advisors Committee
11:30 – 12:45 CJD Foundation Board of Directors Meeting
*This meeting is open only to the members of the CJD Foundation Board of Directors
1:00 – 3:00 Familial Prion Disease Meeting
*This meeting is only for families affected by a familial prion disease
Deana Simpson, RN – Chairman
Founder and Director, CJD Insight; Member, CJD Foundation Board of Directors
Macomb, Michigan
Pierluigi Gambetti, MD
Professor, Case Western Reserve University School of Medicine,
Department of Pathology
Director, The National Prion Disease Pathology Surveillance Center;
Medical Director, The CJD Foundation, CJD Foundation Board of Directors
Cleveland, Ohio
Michael Geschwind, MD, PhD
Assistant Adjunct Professor of Neurology, Memory and Aging Center, University of
California at San Francisco
San Francisco, California
Eva Mitrova´, M.D.
National Reference Centre for Prion Diseases,
Research base of Slovak Medical University,
Limbova´ str. 14, 833 03
Bratislava, Slovakia
2
3:15 - 5:15 Bereavement Workshop
Deana Simpson, RN, Co-chairman
Katrina Hallmark, PsyD, Co-chairman
Neuropsychologist, Southwest Medical Center
San Antonio, Texas
Brian Appleby, MD
Lou Ruvo Center for Brain Health, Neurological Institute
Cleveland Clinic Foundation
Cleveland, Ohio
4:30 – 7:30 Registration
6:00 – 7:30 Welcome Reception
Saturday, July 14th
7:00 a.m. Conference Registration
7:00 – 8:00 Continental Breakfast
8:00 – 8:15 Welcome
Florence Kranitz
President, The CJD Foundation; Co-Chairman, The CJD International Support Alliance
Akron, Ohio
8:15 – 8:40 Memorial Service
Co-Chairmen: Pierluigi Gambetti, MD
Florence Kranitz
Keynote Address
8:40 - 9:10 Byron Caughey, PhD
New Ways of Prion Disease Detection and Diagnosis
Senior Investigator
Chief, TSE/prion Biochemistry Section
Laboratory of Persistent Viral Diseases
NIH/NIAID Rocky Mountain Laboratories
Hamilton, Montana
9:10 – 9:15 Q&A
3
New Discoveries
9:15 – 9:35 Emiliano Biasini, PhD
Novel Anti-Prion Compounds Identified by Targeting the Functional Activity of PrPC
Instructor, Harris Laboratory, Boston University
Boston, Massachusetts
Recipient of the Carlton Madderly Wilson and CJD Foundation Research Grant, 2011
9:35 - 9:40 Q&A
9:40 – 10:00 Edward Málaga-Trillo, Ph.D.
The Use of Zebra Fish to Study PrP Function and the Molecular Basis of Neurodegeneration
University of Konstanz, Department of Biology, Prion Biology Group
Konstanz, Germany
10:00 – 10:05 Q&A
10:05 – 10:15 Coffee Break
10:15 - 10:35 Neil Cashman, MD, FRCP(C), FCAHS
Understanding CJD Leads to New Treatments for ALS
Professor & Scientific Director, PrioNet Canada, a Network of Centres of Excellence
Vancouver, British Columbia
10:35 – 10:40 Q&A
10:40 - 11:00 Glenn Telling, PhD
The Spread of Chronic Wasting Disease and the Alteration of the Species Barrier by In Vitro Amplification
Director and Professor, Prion Research Center
Department of Microbiology, Immunology & Pathology Colorado State University
Fort Collins, Colorado
11:00 - 11:05 Q&A
11:05 - 11:25 Eva Mitrova´, M.D.
The Role of Anticipation in the Incidence of CJD with the Mutation E200K
11:25 - 11:30 Q&A
11:30 - 12:45 Lunch
4
Surveillance
12:45 - 1:05 Robert G. Will, MD, FRCP(E)
European Surveillance Overview
Professor of Neurology, Personal Chair in Clinical Neurology, University of Edinburgh;
Consultant Neurologist, Western General Hospital; Former Director and Founder,
National CJD Surveillance Unit
Edinburgh, United Kingdom
1:05 - 1:10 Q&A
1:10 - 1:30 Pierluigi Gambetti, MD
U.S. Prion Disease Surveillance and the Importance of Autopsy; Tests Conducted
1:30 - 1:35 Q&A
1:35 – 1:55 Daniel Krewski, MHA, MSc, PhD
Summary
Professor and Director of the R. Samuel McLaughlin Centre for Population Health Risk Assessment, University of Ottawa
Ottawa, Ontario
1:55 - 2:00 Q&A
2:00 - 2:10 Coffee Break
2:10 - 4:15 Moving Round Tables
7:00 – 7:30 Reception
7:30 Banquet
Sunday, July 15th
Co-Chairmen: Ruthie George Lori Nusbaum
7:30 – 8:45 Continental Breakfast
9:00 – 9:15 Mark Goldfarb
CJDF Update
Chairman, Board of Directors, The CJD Foundation
Akron, Ohio
5
Ruthie George
Treasurer, Board of Directors, The CJD Foundation
Akron, Ohio
9:15 - 9:30 Lori Nusbaum
Questionnaire Report
Associate Director, The CJD Foundation
Akron, Ohio
9:30 - 9:35 Q&A
9:35 – 10:05 Bradley Kalinsky, MD & Amanda Baxley Kalinsky, RN, BSN
In Vitro Options, A Personal Story
Nashville, Tennessee
10:05 – 10:10 Q&A
10:10 – 11:20 Panel Presentations
Joe Abrams, MPH
Centers for Disease Control and Prevention
NCEZID/DHCPP
Atlanta, Georgia
David Asher, MD
US Food and Drug Administration
Chief and Supervisory Medical Officer in the Laboratory of Bacterial and Transmissible
Spongiform Encephalopathy Agents within FDA’s Division of Emerging and
Transfusion-Transmitted Diseases, Office of Blood Research and Review,
Center for Biologics Evaluation and Research (CBER).
Rockville, Maryland
Marie Kassai RN, BSN, MPH, CIC
On behalf of World Health Organization
Infection Preventionist
Passaic, New Jersey
Christopher A. Waldrop, MPH
Consumer Federation of America
Director, Food Policy Institute
Washington, DC
11:20 - 11:30 Q&A
11:30 - 12:45 Lunch
6
Support Organization Reports
12:45 – 2:15 CJD International Support Alliance Panel Presentation
Introduction, Florence Kranitz & Suzanne Solvyns
Co Chairmen, The CJD International Support Network
Suzanne Solvyns
CJD Support Group Network, Australia
Director, CJD Support Group Network,
Co chairman the CJD International Support Group Network
Sydney NSW, Australia
David Ralston
CJD Support Group Network, Australia
Chair, CJD Support Group Network
Sydney NSW, Australia
Roberto Borgis
Associazione Italiana Encefalopatie da Prioni (A.I.En.P.), Italy
President, A.I.En.P.
Torino, Italy
J.B. Matthieu
MCJ-HCC, France
President, MCJ-HCC
Paris, France
Professor Richard Knight, MD, FRCP(E)
CJD Support Network, U.K.
Medical Director, CJD Support Network, U.K.
Edinburgh, Scotland
Victor Sanchez, MD, PhD
The CJD Foundation, Mexico
Director
Founder and Director, Mexican Survellance
Guadalajara, Mexcio
2:15 – 2:30 Coffee Break
2:30 – 4:15 Advocacy Training
Monday, July 16th
Capitol Hill Visits
http://www.cjdfoundation.org/pdfs/2012conferenceschedule.pdf
also, be warned, these folks don’t like questions being asked that are not screened.
“At the CJD Foundation conference in 2000 our members were accused of 'attacking' the researchers in the question and answer session because of the questions we were asking”.
speaking about that CJD Questionnaire the CJD Foundation and CWRU Gambetti et al are so proud of.
some folks here might want to review some old history about that, and others here might wish I would forget.
not going to happen. ...
Subject: Re: Tracie CJD Foundation
Date: Tue, 5 Nov 2002 15:09:29 -0500
From: "Tracie Kedzierski"
To: "Terry S. Singeltary Sr."
References: <3DC730DF.8010308@wt.net><018601c284f7$27a04a80$0a64a8c0@newportrentalguide .com><3DC80F1A.3090808@wt.net><000901c28502$fac15c00$0a64a8c0@newportrentalguide .com><3DC81FCA.1040403@wt.net>
Terry,
Oh no....I've gone and pissed you off (ha ha)I just find it better to speak...so that nothing I write is misinterpreted. It is very important to me that you understand the conflict, the confusion, etc so can I call you or not? My dime ?
Tracie
----- Original Message -----
From: "Terry S. Singeltary Sr."
To: "Tracie Kedzierski"
Sent: Tuesday, November 05, 2002 2:45 PM
Subject: Re: Tracie CJD Foundation
> either mail me your explination or forget the it...TSS
>> Tracie Kedzierski wrote:
>> > Terry,
The only problem is that having it on our message board conflicts with the information I have on our home page about the surveillance project and the report form I send out to the families. -----it is confusing. In fact.. I'm sorry but we (The Foundation) have to pull it off. I need to talk to you about this and share a number of goals the "new" Foundation has Can I call you? Please email me your number.... Tracie
> > Original Message -----
> > From: "Terry S. Singeltary Sr." > >
To: "Tracie Kedzierski"
Sent: Tuesday, November 05, 2002 1:34 PM
Subject: Re: Tracie CJD Foundation
hi Tracie,
doing fine, thank you. about the questionnaire? by no means am i trying to step on Dr Gambetti's toes here. i think there is more to it than just reporting a case. we _must_ find the source and route of spordic CJDs, and i think a great deal of it will be from the medical/surgical arena. i just want a questionnaire made up for _all_ victims of human TSEs in the USA in _every_ state, and i want it reportable in _every_ state. i am turning the heat up. ****, i'm getting old and grey, i want to see it done before i die. will be sending this out to many media and papers and requesting them to turn the heat up on the Gov. you will be able to keep up with the ones coming through the voice and if i get some that have not come through the list, i will pass on to Dr. Gambetti if he likes. i respect Dr. Gambetti very much and would do nothing to hender his work or yours. i just think that this is too important of a matter not to have one. and i think by turning the heat up, getting to the media and pressing there buttons a bit, just might help this get done a bit faster... hope so anyway...
kindest regards,
terry
>>Tracie Kedzierski wrote:> >>> >>>
Hi Terry,
How are you? I'm just curious about your Questionnaire ?
It just was posted on the Foundation's Message Board without any introduction... and I was a bit concerned as it may cause some confusion with the Surveillance Project I'm doing via the Foundation for Dr Gambetti. Could you let me know?
Tracie
Greetings again Voice,
just what is the _new_ CJD Foundations goals witha CJD Questionnaire that asks _no_
questions about soure/route of the six variants of sporadic CJDs???
=================================================
i am reminded of a few things deep throat told me years ago;
=================================================
The most frightening thing I have read all day is the report of Gambetti's finding of a new strain of sporadic cjd in young people......... Dear God, what in the name of all that is holy is that!!! If the US has different strains of scrapie..... why???? than the UK... then would the same mechanisms that make different strains of scrapie here make different strains of BSE... if the patterns are different in sheep and mice for scrapie..... could not the BSE be different in the cattle, in the mink, in the humans....... I really think the slides or tissues and everything from these young people with the new strain of sporadic cjd should be put up to be analyzed by many, many experts in cjd........ bse..... scrapie
Scrape the damn slide and put it into mice..... wait..... chop up the mouse brain and and spinal cord........ put into some more mice..... dammit amplify the thing and start the damned research..... This is NOT rocket science... we need to use what we know and get off our butts and move.... the whining about how long everything takes..... well it takes a whole lot longer if you whine for a year and then start the research!!!
Not sure where I read this but it was a recent press release or something like that: I thought I would fall out of my chair when I read about how there was no worry about infectivity from a histopath slide or tissues because they are preserved in formic acid, or formalin or formaldehyde..... for God's sake........ Ask any pathologist in the UK what the brain tissues in the formalin looks like after a year....... it is a big fat sponge... the agent continues to eat the brain ...... you can't make slides anymore because the agent has never stopped........ and the old slides that are stained with Hemolysin and Eosin...... they get holier and holier and degenerate and continue... what you looked at 6 months ago is not there........ Gambetti better be photographing every damned thing he is looking at.....
Okay, you need to know. You don't need to pass it on as nothing will come of it and there is not a damned thing anyone can do about it. Don't even hint at it as it will be denied and laughed at.......... USDA is gonna do as little as possible until there is actually a human case in the USA of the nvcjd........ if you want to move this thing along and shake the earth.... then we gotta get the victims families to make sure whoever is doing the autopsy is credible, trustworthy, and a saint with the courage of Joan of Arc........ I am not kidding!!!! so, unless we get a human death from EXACTLY the same form with EXACTLY the same histopath lesions as seen in the UK nvcjd........ forget any action........ it is ALL gonna be sporadic!!! And, if there is a case....... there is gonna be every effort to link it to international travel, international food, etc. etc. etc. etc. etc. They will go so far as to find out if a sex partner had ever traveled to the UK/europe, etc. etc. .... It is gonna be a long, lonely, dangerous twisted journey to the truth. They have all the cards, all the money, and are willing to threaten and carry out those threats.... and this may be their biggest downfall...
Thanks as always for your help. (Recently had a very startling revelation from a rather senior person in government here.......... knocked me out of my chair........ you must keep pushing. If I was a power person.... I would be demanding that there be at least a million bovine tested as soon as possible and agressively seeking this disease. The big players are coming out of the wood work as there is money to be made!!!
In short: "FIRE AT WILL"!!! for the very dumb.... who's "will"! "Will be the burden to bare if there is any coverup!"
again it was said years ago and it should be taken seriously.... BSE will NEVER be found in the US!
As for the BSE conference call... I think you did agreat service to freedom of information and making some people feign integrity... I find it scary to see that most of the "experts" are employed by the federal government or are supported on the "teat" of federal funds. A scary picture! I hope there is a confidential panel organized by the new government to really investigate this thing.
You need to watch your back........ but keep picking at them....... like a buzzard to the bone... you just may get to the truth!!! (You probably have more support than you know. Too many people are afraid to show you or let anyone else know. I have heard a few things myself... you ask the questions that everyone else is too afraid to ask.)
================================================
greetings again voice,
then i remind everyone to read this;
'As implied in the Inset 25 we must not assume that transmission of BSE to other species will invariably present pathology typical of a scrapie-like disease.'
http://www.bseinquiry.gov.uk/files/yb/1991/01/04004001.pdf
TSS
#################### https://lists.aegee.org/bse-l.html ####################
FOR THOSE THAT MIGHT BE INTERESTED, see the CJD Foundation CWRU prion unit old original CJD Questionnaire, compared to the one I proposed here (others on cjdvoice helped add questions). the CJD Foundation and cwru et al were very upset about the CJD Questionnaire and the questions pertaining to _all_ potential routes and sources of the TSE prion disease I put on the web, and some were filling out and sending to me. the CJD questionnaire at the time the CJD foundation cwru Gambetti prion unit had, ask NO questions pertaining to route and source. all it ask was how CJD was diagnosed, and by whom, where, etc. NOTHING about route and source. we hounded them and hounded them about this. once they proposed one that did ask a few questions, years later, they then did not give it out to everyone. they picked and chose who got one. now, today, I don’t know how the CJD Questionnaire is handled. they were so proud of what WE did (they forgot about that, or refused to acknowledge that), they then went on to congratulate everyone that _opposed_ the CJD Questionnaire at first, the ones that refused to ask any questions pertaining to potential routes and sources, at one of the CJD Foundations conferences. nope, they did not like that at all. but here is the history on that. some may not like this, but I will bring it up every year I have a breath of air left in me. Cele was right all along. ...
Friday, November 30, 2007
CJD QUESTIONNAIRE USA CWRU AND CJD FOUNDATION
http://cjdquestionnaire.blogspot.com/2007/11/cjd-questionnaire.html
http://cjdquestionnaire.blogspot.com/
Subject: Re: CJD FOUNDATION CONFERENCE A STEP BACKWARD, bending over to be non-controversial (amen)
Date: Mon, 20 Jan 2003 12:17:10 –0600
From: "Terry S. Singeltary Sr."
To: John Stauber
CC: bloodcjd
References:
hi john,
John Stauber wrote:
> --- drew my attention to the agenda for the upcoming CJD
> Foundation conference. Beth Willaims is doing a presentation
> on 'does CWD cause CJD'. The conference agenda seems a big
> step backwards, bending over to be non-controversial. This is
> typical with what happens to "disease victim" organizations,
> unforunately.
my friend, i could not agree more. just another...SNIP...END...TSS
kind regards,
terry
VARIABLY PROTEASE-SENSITVE PRIONOPATHY IS TRANSMISSIBLE ...price of prion poker goes up again $
OR-10 15:25 - 15:40 VARIABLY PROTEASE-SENSITIVE PRIONOPATHY IS TRANSMISSIBLE IN BANK VOLES Nonno
http://www.prion2012.com/program/final-program
I believe it was Gambetti et al that coined this term sporadic FFI, from some conspicuous sub-type of sporadic CJD possibly? seems they could not tie it to a true FFI by diagnostic standards to date, so it was then termed a sFFI, confusing matters even worse. ...
A subtype of sporadic prion disease mimicking fatal familial insomnia
http://www.neurology.org/cgi/content/abstract/52/9/1757?ck=nck
THIS seems to raise more questions than answers, confusing the TSEs even worse.
WHAT is sporadic CJD, and how many sub-types and atypical strains, phenotypes etc. will there be, arising from nothing. a spontaneous happening of sorts???
i think not. ...tss
http://sporadicffi.blogspot.com/
Wednesday, October 27, 2010
A novel variant of human disease with a protease-sensitive prion protein and heterozygosity methionine/valine at codon 129: Case report
snip...
Genetic findings
No mutations were found in the open reading frame after sequencing the prion protein gene (PRNP). A heterozygosis methionine valine (MV) was observed in codon 129.
snip...
http://creutzfeldt-jakob-disease.blogspot.com/2010/10/novel-variant-of-human-disease-with.html
Thursday, July 10, 2008
A Novel Human Disease with Abnormal Prion Protein Sensitive to Protease update July 10, 2008
Although several subjects had family histories of dementia, no mutations were found in the PrP gene open reading frame.
http://cjdmadcowbaseoct2007.blogspot.com/2008/07/novel-human-disease-with-abnormal-prion.html
Thursday, July 10, 2008
A New Prionopathy update July 10, 2008
http://cjdmadcowbaseoct2007.blogspot.com/2008/07/new-prionopathy-update-july-10-2008.html
***+++***
Thursday, July 10, 2008
A Novel Human Disease with Abnormal Prion Protein Sensitive to Protease update July 10, 2008
Friday, June 20, 2008
http://cjdmadcowbaseoct2007.blogspot.com/2008/07/novel-human-disease-with-abnormal-prion.html
Here we go folks. AS predicted. THIS JUST OUT !
Tuesday, August 03, 2010
Variably protease-sensitive prionopathy: A new sporadic disease of the prion protein
http://creutzfeldt-jakob-disease.blogspot.com/2010/08/variably-protease-sensitive-prionopathy.html
Monday, August 9, 2010
Variably protease-sensitive prionopathy: A new sporadic disease of the prion protein or just more PRIONBALONEY ?
snip...see full text ;
http://transmissiblespongiformencephalopathy.blogspot.com/2011/05/variably-protease-sensitive-prionopathy.html
http://prionopathy.blogspot.com/2011/05/variably-protease-sensitive-prionopathy.html
O.K. let's compare some recent cases of this prionpathy in other countries besides Gambetti's first 10 recently, that he claims is a spontaneous event, from a genetic disorder, that is not genetic, but sporadic, that is related to no animal TSE in North America, or the world. ...
http://prionunitusaupdate2008.blogspot.com/2010/08/variably-protease-sensitive-prionopathy.html
Wednesday, October 27, 2010
A novel variant of human disease with a protease-sensitive prion protein and heterozygosity methionine/valine at codon 129: Case report
http://creutzfeldt-jakob-disease.blogspot.com/2010/10/novel-variant-of-human-disease-with.html
Sunday, August 09, 2009
CJD...Straight talk with...James Ironside...and...Terry Singeltary... 2009
http://creutzfeldt-jakob-disease.blogspot.com/2009/08/cjdstraight-talk-withjames.html
Tuesday, August 18, 2009
BSE-The Untold Story - joe gibbs and singeltary 1999 – 2009
http://madcowusda.blogspot.com/2009/08/bse-untold-story-joe-gibbs-and.html
====================================
The familial mutations, Gajdusek proposed, lowered the barrier to such accidental conversion. "Thus," he wrote in 1996, "with these mutations, this ordinarily rare event becomes a ... dominant inherited trait." But Weissmann's qualification still remained to be refuted: the mutations might simply allow easier entry to a lurking virus. ...page 202 Deadly Feast
===================================
something to think about for sure.
but i interpret this as (1st not the gold standard, just my opinion;-), as because of certain gene mutations, one or a family, would be more susceptible to the many different strains of TSE, and the many different proven routes and sources, (which will cause different symptoms, different incubation periods from onset of clinical symptoms to death, different parts of the brain infected, etc.). in other words, it's NOT the gene mutation that CAUSES the disease, but the fact that it makes you more SUSCEPTIBLE, to the TSEs from the surrounding environment, and PLUS accumulation, i think this plays a critical role. maybe there is a one dose scenario, but i think there is more of the 'accumulators' that go clinical, than the 'one dose'. and what is the threshold to sub-clinical to clinical ?
anyway, just pondering out loud here.
also, for anyone interested, there are some studies with links to follow here ;
http://sporadicffi.blogspot.com/
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 $$$
http://madcowtesting.blogspot.com/2009/07/us-emergency-bovine-spongiform.html
LET'S take a closer look at this new prionpathy or prionopathy, and then let's look at the g-h-BSEalabama mad cow. This new prionopathy in humans? the genetic makeup is IDENTICAL to the g-h-BSEalabama mad cow, the only _documented_ mad cow in the world to date like this, ......wait, it get's better. this new prionpathy is killing young and old humans, with LONG DURATION from onset of symptoms to death, and the symptoms are very similar to nvCJD victims, OH, and the plaques are very similar in some cases too, bbbut, it's not related to the g-h-BSEalabama cow, WAIT NOW, it gets even better, the new human prionpathy that they claim is a genetic TSE, has no relation to any gene mutation in that family. daaa, ya think it could be related to that mad cow with the same genetic make-up ??? there were literally tons and tons of banned mad cow protein in Alabama in commerce, and none of it transmitted to cows, and the cows to humans there from ??? r i g h t $$$ ALABAMA MAD COW g-h-BSEalabama In this study, we identified a novel mutation in the bovine prion protein gene (Prnp), called E211K, of a confirmed BSE positive cow from Alabama, United States of America. This mutation is identical to the E200K pathogenic mutation found in humans with a genetic form of CJD. This finding represents the first report of a confirmed case of BSE with a potential pathogenic mutation within the bovine Prnp gene. We hypothesize that the bovine Prnp E211K mutation most likely has caused BSE in "the approximately 10-year-old cow" carrying the E221K mutation.
http://www.plospathogens.org/article/info%3Adoi%2F10.1371%2Fjournal.ppat.1000156
http://www.plospathogens.org/article/fetchObjectAttachment.action?uri=info%3Adoi%2F10.1371%2Fjournal.ppat.1000156&representation=PDF
Saturday, August 14, 2010
BSE Case Associated with Prion Protein Gene Mutation (g-h-BSEalabama) and VPSPr PRIONPATHY (see mad cow feed in COMMERCE IN ALABAMA...TSS)
http://prionpathy.blogspot.com/2010/08/bse-case-associated-with-prion-protein.html
her healthy calf also carried the mutation (J. A. Richt and S. M. Hall PLoS Pathog. 4, e1000156; 2008).
This raises the possibility that the disease could occasionally be genetic in origin. Indeed, the report of the UK BSE Inquiry in 2000 suggested that the UK epidemic had most likely originated from such a mutation and argued against the scrapierelated assumption. Such rare potential pathogenic PRNP mutations could occur in countries at present considered to be free of BSE, such as Australia and New Zealand. So it is important to maintain strict surveillance for BSE in cattle, with rigorous enforcement of the ruminant feed ban (many countries still feed ruminant proteins to pigs). Removal of specified risk material, such as brain and spinal cord, from cattle at slaughter prevents infected material from entering the human food chain. Routine genetic screening of cattle for PRNP mutations, which is now available, could provide additional data on the risk to the public. Because the point mutation identified in the Alabama animals is identical to that responsible for the commonest type of familial (genetic) CJD in humans, it is possible that the resulting infective prion protein might cross the bovine-human species barrier more easily. Patients with vCJD continue to be identified. The fact that this is happening less often should not lead to relaxation of the controls necessary to prevent future outbreaks. Malcolm A. Ferguson-Smith Cambridge University Department of Veterinary Medicine, Madingley Road, Cambridge CB3 0ES, UK e-mail: mailto:maf12%40cam.ac.uk Jürgen A. Richt College of Veterinary Medicine, Kansas State University, K224B Mosier Hall, Manhattan, Kansas 66506-5601, USA NATURE|Vol 457|26 February 2009
http://www.nature.com/nature/journal/v457/n7233/full/4571079b.html
SEE FULL TEXT OF ALL THIS HERE ;
2009 UPDATE ON ALABAMA AND TEXAS MAD COWS 2005 and 2006
http://bse-atypical.blogspot.com/2006/08/bse-atypical-texas-and-alabama-update.html
Saturday, June 25, 2011
Transmissibility of BSE-L and Cattle-Adapted TME Prion Strain to Cynomolgus Macaque
"BSE-L in North America may have existed for decades"
http://transmissiblespongiformencephalopathy.blogspot.com/2011/06/transmissibility-of-bse-l-and-cattle.html
Over the next 8-10 weeks, approximately 40% of all the adult mink on the farm died from TME.
snip...
The rancher was a ''dead stock'' feeder using mostly (>95%) downer or dead dairy cattle...
http://web.archive.org/web/20030516051623/http://www.bseinquiry.gov.uk/files/mb/m09/tab05.pdf
Sunday, June 26, 2011
Risk Analysis of Low-Dose Prion Exposures in Cynomolgus Macaque
http://transmissiblespongiformencephalopathy.blogspot.com/2011/06/risk-analysis-of-low-dose-prion.html
Monday, September 26, 2011
L-BSE BASE prion and atypical sporadic CJD
http://bse-atypical.blogspot.com/2011/09/l-bse-base-prion-and-atypical-sporadic.html
for more, see full text here ;
Monday, September 26, 2011
Variably Protease-Sensitive Prionopathy, Prionpathy, Prionopathy, FFI, GSS, gCJD, hvCJD, sCJD, TSE, PRION, update 2011
http://prionopathy.blogspot.com/2011/09/variably-protease-sensitive-prionopathy.html
DEEP THROAT TO TSS 2000-2001 (take these old snips of emails with how ever many grains of salt you wish. ...tss)
The most frightening thing I have read all day is the report of Gambetti's finding of a new strain of sporadic cjd in young people...Dear God, what in the name of all that is holy is that!!! If the US has different strains of scrapie.....why????than the UK...then would the same mechanisms that make different strains of scrapie here make different strains of BSE...if the patterns are different in sheep and mice for scrapie.....could not the BSE be different in the cattle, in the mink, in the humans.......I really think the slides or tissues and everything from these young people with the new strain of sporadic cjd should be put up to be analyzed by many, many experts in cjd........bse.....scrapie Scrape the damn slide and put it into mice.....wait.....chop up the mouse brain and and spinal cord........put into some more mice.....dammit amplify the thing and start the damned research.....This is NOT rocket science...we need to use what we know and get off our butts and move....the whining about how long everything takes.....well it takes a whole lot longer if you whine for a year and then start the research!!! Not sure where I read this but it was a recent press release or something like that: I thought I would fall out of my chair when I read about how there was no worry about infectivity from a histopath slide or tissues because they are preserved in formic acid, or formalin or formaldehyde.....for God's sake........ Ask any pathologist in the UK what the brain tissues in the formalin looks like after a year.......it is a big fat sponge...the agent continues to eat the brain ......you can't make slides anymore because the agent has never stopped........and the old slides that are stained with Hemolysin and Eosin......they get holier and holier and degenerate and continue...what you looked at 6 months ago is not there........Gambetti better be photographing every damned thing he is looking at.....
Okay, you need to know. You don't need to pass it on as nothing will come of it and there is not a damned thing anyone can do about it. Don't even hint at it as it will be denied and laughed at.......... USDA is gonna do as little as possible until there is actually a human case in the USA of the nvcjd........if you want to move this thing along and shake the earth....then we gotta get the victims families to make sure whoever is doing the autopsy is credible, trustworthy, and a saint with the courage of Joan of Arc........I am not kidding!!!! so, unless we get a human death from EXACTLY the same form with EXACTLY the same histopath lesions as seen in the UK nvcjd........forget any action........it is ALL gonna be sporadic!!!
And, if there is a case.......there is gonna be every effort to link it to international travel, international food, etc. etc. etc. etc. etc. They will go so far as to find out if a sex partner had ever traveled to the UK/europe, etc. etc. .... It is gonna be a long, lonely, dangerous twisted journey to the truth. They have all the cards, all the money, and are willing to threaten and carry out those threats....and this may be their biggest downfall...
Thanks as always for your help. (Recently had a very startling revelation from a rather senior person in government here..........knocked me out of my chair........you must keep pushing. If I was a power person....I would be demanding that there be a least a million bovine tested as soon as possible and agressively seeking this disease. The big players are coming out of the woodwork as there is money to be made!!! In short: "FIRE AT WILL"!!! for the very dumb....who's "will"! "Will be the burden to bare if there is any coverup!"
again it was said years ago and it should be taken seriously....BSE will NEVER be found in the US! As for the BSE conference call...I think you did a great service to freedom of information and making some people feign integrity...I find it scary to see that most of the "experts" are employed by the federal government or are supported on the "teat" of federal funds. A scary picture! I hope there is a confidential panel organized by the new government to really investigate this thing.
You need to watch your back........but keep picking at them.......like a buzzard to the bone...you just may get to the truth!!! (You probably have more support than you know. Too many people are afraid to show you or let anyone else know. I have heard a few things myself... you ask the questions that everyone else is too afraid to ask.)
END...TSS
IN CONFIDENCE
Perceptions of unconventional slow virus in the USA
GAH WELLS
Report of a visit to the U.S.A. April-May 1989
3. Prof. A Robertson gave a brief account of BSE. The US approach was to accord it a very low profile indeed.
Dr. A Thiermann showed the picture in the ''Independent'' with cattle being incinerated and thought this was a fantical incident to be avoided in the USA AT ALL COSTS.
http://web.archive.org/web/20060307063531/http://www.bseinquiry.gov.uk/files/mb/m11b/tab01.pdf
and they meant it !
Monday, March 19, 2012
Infectivity in Skeletal Muscle of Cattle with Atypical Bovine Spongiform Encephalopathy
PLoS One. 2012; 7(2): e31449.
http://transmissiblespongiformencephalopathy.blogspot.com/2012/03/infectivity-in-skeletal-muscle-of.html
Saturday, March 5, 2011
MAD COW ATYPICAL CJD PRION TSE CASES WITH CLASSIFICATIONS PENDING ON THE RISE IN NORTH AMERICA
http://transmissiblespongiformencephalopathy.blogspot.com/2011/03/mad-cow-atypical-cjd-prion-tse-cases.html
Sunday, February 12, 2012
National Prion Disease Pathology Surveillance Center Cases Examined1 (August 19, 2011) including Texas
http://transmissiblespongiformencephalopathy.blogspot.com/2012/02/national-prion-disease-pathology.html
kind regards, terry
Wednesday, March 28, 2012
VARIABLY PROTEASE-SENSITVE PRIONOPATHY IS TRANSMISSIBLE, price of prion poker goes up again $
http://prionopathy.blogspot.com/2012/03/variably-protease-sensitve-prionopathy.html
TSS
Labels: CJD FOUNDATION FAMILIAL FAMILY AFFAIR CONFERENCE 2012
posted by Terry S. Singeltary Sr. at
6:16 PM
