Evaluation of the Human Transmission Risk of an Atypical BSE

[flounder]

Thursday, January 31, 2008

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

J. Virol. doi:10.1128/JVI.02561-07
Copyright (c) 2008, American Society for Microbiology and/or the Listed Authors/Institutions.
All Rights Reserved.



Thursday, January 31, 2008
Evaluation of the Human Transmission Risk of an Atypical Bovine Spongiform Encephalopathy Prion Strain
J. Virol. doi:10.1128/JVI.02561-07
Copyright (c) 2008, American Society for Microbiology and/or the Listed Authors/Institutions. All Rights Reserved.

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

Qingzhong Kong*, Mengjie Zheng, Cristina Casalone, Liuting Qing, Shenghai Huang, Bikram Chakraborty, Ping Wang, Fusong Chen, Ignazio Cali, Cristiano Corona, Francesca Martucci, Barbara Iulini, Pierluigi Acutis, Lan Wang, Jingjing Liang, Meiling Wang, Xinyi Li, Salvatore Monaco, Gianluigi Zanusso, Wen-Quan Zou, Maria Caramelli, and Pierluigi Gambetti*
Department of Pathology, Case Western Reserve University, Cleveland, OH 44106, USA; CEA, Istituto Zooprofilattico Sperimentale, 10154 Torino, Italy; Department of Neurological and Visual Sciences, University of Verona, 37134 Verona, Italy



* To whom correspondence should be addressed. Email: [email protected]. [email protected].


Abstract


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


http://jvi.asm.org/cgi/content/abstract/JVI.02561-07v1?papetoc


Thursday, January 31, 2008

SPONGIFORM ENCEPHALOPATHY ADVISORY COMMITTEE Draft minutes of the 99th
meeting held on 14th December 2007


snip...


snip...


ITEM 8 – PUBLIC QUESTION AND ANSWER SESSION


40. The Chair explained that the purpose of the question and answer
session was to give members of the public an opportunity to ask
questions related to the work of SEAC. Mr Terry Singeltary
(Texas, USA) had submitted a question prior to the meeting,
asking: "With the Nor-98 now documented in five different states so
far in the USA in 2007, and with the two atypical BSE H-base


13
© SEAC 2007


cases in Texas and Alabama, with both scrapie and chronic
wasting disease (CWD) running rampant in the USA, is there any
concern from SEAC with the rise of sporadic CJD in the USA from
''unknown phenotype'', and what concerns if any, in relations to
blood donations, surgery, optical, and dental treatment, do you
have with these unknown atypical phenotypes in both humans and
animals in the USA? Does it concern SEAC, or is it of no concern
to SEAC? Should it concern USA animal and human health
officials?"


41. A member considered that this question ............


snip... please see full text, sources, and comments here ;


http://seac992007.blogspot.com/2008/01/spongiform-encephalopathy-advisory.html


APHIS-2006-0041-0006 TSE advisory committee for the meeting December 15,
2006


http://www.regulations.gov/fdmspublic/ContentViewer?objectId=09000064801f3413&disposition=attachment&contentType=msw8


[Docket No. FSIS-2006-0011] FSIS Harvard Risk Assessment of Bovine
Spongiform Encephalopathy (BSE)


http://www.fsis.usda.gov/OPPDE/Comments/2006-0011/2006-0011-1.pdf



[Docket No. 03-025IFA] FSIS Prohibition of the Use of Specified Risk Materials for Human Food and Requirement for the Disposition of Non-Ambulatory Disabled Cattle


http://www.fsis.usda.gov/OPPDE/Comments/03-025IFA/03-025IFA-2.pdf



Attachment to Singletary comment

January 28, 2007


Greetings APHIS,


I would kindly like to submit the following to ;


BSE; MRR; IMPORTATION OF LIVE BOVINES AND PRODUCTS DERIVED FROM BOVINES
[Docket No. APHIS-2006-0041] RIN 0579-AC01



[Federal Register: January 9, 2007 (Volume 72, Number 5)]
[Proposed Rules]
[Page 1101-1129]
From the Federal Register Online via GPO Access [wais.access.gpo.gov]
[DOCID:fr09ja07-21]



http://www.regulations.gov/fdmspublic/component/main?main=DocumentDetail&o=09000064801f8152



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


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



Diagnosis and Reporting of Creutzfeldt-Jakob Disease

Singeltary, Sr et al. JAMA.2001; 285: 733-734.


Diagnosis and Reporting of Creutzfeldt-Jakob Disease

Since this article does not have an abstract, we have provided the first 150 words of the full text and any section headings.


To the Editor:

In their Research Letter, Dr Gibbons and colleagues1 reported that the annual US death rate due to Creutzfeldt-Jakob disease (CJD) has been stable since 1985. These estimates, however, are based only on reported cases, and do not include misdiagnosed or preclinical cases. It seems to me that misdiagnosis alone would drastically change these figures. An unknown number of persons with a diagnosis of Alzheimer disease in fact may have CJD, although only a small number of these patients receive the postmortem examination necessary to make this diagnosis. Furthermore, only a few states have made CJD reportable. Human and animal transmissible spongiform encephalopathies should be reportable nationwide and internationally.

Terry S. Singeltary, Sr
Bacliff, Tex



1. Gibbons RV, Holman RC, Belay ED, Schonberger LB. Creutzfeldt-Jakob disease in the United States: 1979-1998. JAMA. 2000;284:2322-2323. FREE FULL TEXT


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



PDF]Freas, William TSS SUBMISSION
File Format: PDF/Adobe Acrobat -Page 1. J Freas, William From: Sent:
To:
Subject:
Terry S. SingeltarySr. [[email protected]]
Monday, January 08,200l 3:03 PM freas ...

http://www.fda.gov/ohrms/dockets/ac/01/slides/3681s2_09.pdf


Asante/Collinge et al, that BSE transmission to the 129-methionine genotype can lead to an alternate phenotype that is indistinguishable from type 2 PrPSc, the commonest _sporadic_ CJD;

http://www.fda.gov/ohrms/dockets/ac/03/slides/3923s1_OPH.htm


DER SPIEGEL (9/2001) - 24.02.2001 (9397 Zeichen)USA: Loch in der MauerDie BSE-Angst erreicht Amerika: Trotz strikter Auflagen gelangte in Texas verbotenes Tiermehl ins Rinderfutter - die Kontrollen der Aufsichtsbehördensind lax.Link auf diesen Artikel im Archiv:http://service.spiegel.de/digas/find?DID=18578755


"Its as full of holes as Swiss Cheese" says Terry Singeltary of the FDA regulations. ...


http://service.spiegel.de/digas/servlet/find/DID=18578755


2 January 2000

British Medical Journal

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

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


15 November 1999

British Medical Journal

vCJD in the USA * BSE in U.S.


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


please notice Texas 2006 ;

† Confirmed in United Kingdom and reported to Texas Department of State
Health Services
through Centers for Disease Control and Prevention.


Only 1 case of variant
CJD has ever been diagnosed in Texas.
The patient was a former resident of the
United Kingdom, where the exposure
was likely to have occurred. Texas has a
population of 23 million, and since the
national rate of sporadic CJD is about 1
per million, it is expected that
approximately 23 cases of CJD would
occur each year in the state. Therefore,
it is believed that CJD is currently underreported
in Texas. ...END...TSS


also see ;


http://www.dshs.state.tx.us/idcu/disease/creutzfeldt-jakob/data/


Creutzfeldt-Jakob Disease in Northeast Texas,

J.A. Rawlings,*1 K.A.
Hendricks1, O.M. Nuno1, D.A. Brown1, D.A. Evans2, Texas Department of
Health, 1Austin and 2Tyler, Texas


Creutzfeldt-Jacob Disease (CJD), a transmissible spongiform
encephalopathy, is caused by prions composed of proteinaceous material
devoid of nucleic acid. CJD occurs sporadically (generally 1
case/1,000,000 population per year) in older patients (average age of
65) and is characterized by rapidly progressive dementia, accompanied by
severe muscle spasms and incoordination. Death usually occurs within 3
to 12 months (average 7 months). CJD activity in Texas, which has a
population of nearly 19 million, appeared to be typical. The statewide
death rate for 1995 and 1996 was just under 1/1,000,000. In April of
1997, the Texas Department of Health became aware of an increased number
of possible CJD cases in a 23-county area of NE Texas with a population
of just over one million. After review of medical and pathology records,
four patients were identified with definite classic CJD and three were
identified with probable CJD. Dates of death for the eight patients were
from April, 1996 through mid-July 1997. The patients were from 46
through 65 years of age; four were male and three were female. A
case-control study to identify risks for CJD in NE Texas has been initiated.


http://www.jifsan.umd.edu/tse/Rawlings.htm


Division of Neuropathology
Pierluigi Gambetti, M.D.,
Director

-----------------------------------

CASE WESTERN RESERVE UNIVERSITY

March 30, 1998

Dr. Gerald A, Campbell

The University of Texas
Medical Branch at Galveston
Division of Neuropathology
Department of Pathology
Galveston, Texas

Dear Dr Campbell,

We performed Western immunoblot analysis on the frozen tissue from your case
#AU97-435 (our #098-28). The Immunoblot reveals the presence of
protease-resistant prion protein (PrPres) confirming the diagnosis of prion
disease. The immunoblot pattern of PrPres is consistent with the diagnosis
of Creutzfeldt-Jakob disease.

Thank you for referring to us this interesting case.


Sincerely,

Piero Parchi, M.D.

Pierluigi Gambetti, M.D.

PP:sbDivision of Neuropathology

Pierluigi Gambetti, M.D.,

Director

Case Western Reserve University

snip...end


see full text ;


http://cjdtexas.blogspot.com/


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

http://cjdusa.blogspot.com/


Creutzfeldt Jakob Disease

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


Creutzfeldt-Jakob Disease, Prion Protein Gene Codon 129VV, and a Novel PrPSc Type in a Young British Woman


http://creutzfeldt-jakob-disease.blogspot.com/2008/01/creutzfeldt-jakob-disease-prion-protein.html


CJD QUESTIONNAIRE

http://cjdquestionnaire.blogspot.com/


ANIMAL HEALTH REPORT 2006 (BSE h-BASE EVENT IN ALABAMA, Scrapie, and CWD)

http://animalhealthreport2006.blogspot.com/


CREUTZFELDT JAKOB DISEASE MAD COW BASE UPDATE USA

http://cjdmadcowbaseoct2007.blogspot.com/


Friday, January 11, 2008

CJD HUMAN TSE REPORT UK, USA, CANADA, and Mexico JANUARY 2008


http://cjdmadcowbaseoct2007.blogspot.com/2008/01/cjd-human-tse-report-uk-usa-canada-and.html


Friday, January 25, 2008
January 2008 Update on Feed Enforcement Activities to Limit the Spread of BSE

http://madcowspontaneousnot.blogspot.com/2008/01/january-2008-update-on-feed-enforcement.html

http://madcowspontaneousnot.blogspot.com/


BSE BASE MAD COW TESTING TEXAS, USA, AND CANADA

http://madcowtesting.blogspot.com/


NOR-98 ATYPICAL SCRAPIE USA UPDATE AS AT OCT 2007

http://nor-98.blogspot.com/

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


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



http://cjdmadcowbaseoct2007.blogspot.com/2008/01/evaluation-of-human-transmission-risk.html

http://cjdmadcowbaseoct2007.blogspot.com/


TSS

 

[QUESTION]

Flounder am i reading this right - BASE is more transmisible than BSE, thus more dangerous. And in north texas the rate of CJD is 8 per million compared to the normal levels of 1 per million occurance rate in the general population. WOW that is 8 times the normal levels you would expect in the general population. How long until the exponential increase in CJD cases in north texas? Still no BASE/BSE problem in the US. Denial only last so long. :frowns:

 

[Sandhusker]

Flounder am i reading this right - BASE is more transmisible than BSE, thus more dangerous. And in north texas the rate of CJD is 8 per million compared to the normal levels of 1 per million occurance rate in the general population. WOW that is 8 times the normal levels you would expect in the general population. How long until the exponential increase in CJD cases in north texas? Still no BASE/BSE problem in the US. Denial only last so long. :frowns:

What, you're doubting the USDA now? Don't you remember the Ninth Court's decisions (that was widely hailed up there) that said the USDA must be given deference?

 

[QUESTION]

:roll:

 

[Sandhusker]

:roll:

That's kind of what I thought. Once again, you changed your tune when you had to deal with the crap we have to.

 

[QUESTION]

SH :roll: The testing on base virulence was not done by the USDA and the data on cjd was complied by the texas depatment of health from deaths in NE texas. So the USDA had nothing to do with this maybe do some reading :roll:

 

[Sandhusker]

SH :roll: The testing on base virulence was not done by the USDA and the data on cjd was complied by the texas depatment of health from deaths in NE texas. So the USDA had nothing to do with this maybe do some reading :roll:

The USDA claimed we don't have a BSE/BASE problem in this country.

 

[Bill]

SH :roll: The testing on base virulence was not done by the USDA and the data on cjd was complied by the texas depatment of health from deaths in NE texas. So the USDA had nothing to do with this maybe do some reading :roll:

The USDA claimed we don't have a BSE/BASE problem in this country.

So are now admitting you do? Tired of pointing the finger North?

 

[Sandhusker]

SH :roll: The testing on base virulence was not done by the USDA and the data on cjd was complied by the texas depatment of health from deaths in NE texas. So the USDA had nothing to do with this maybe do some reading :roll:

The USDA claimed we don't have a BSE/BASE problem in this country.

So are now admitting you do? Tired of pointing the finger North?

I don't recall ever saying we didn't have BSE down here, Bill. Can you find a quote of mine? It would be foolish to say we don't have it when we're importing it right now.

Would you guys let us know when we are supposed to trust the USDA knows what they're saying and when they don't? What's the formula/clues that you use?

 

[Bill]

The USDA claimed we don't have a BSE/BASE problem in this country.

So are now admitting you do? Tired of pointing the finger North?

I don't recall ever saying we didn't have BSE down here, Bill. Can you find a quote of mine? It would be foolish to say we don't have it when we're importing it right now.

Would you guys let us know when we are supposed to trust the USDA knows what they're saying and when they don't? What's the formula/clues that you use?

Yes you do have BSE although it certainly must have been embarrassing when Phyllis Fong forced you to admit it but then it was on to the US..A-typical strain angle so that still doesn't really count according to some. It sure is odd though how according to you the US is importing BSE yet, other than the Dec 2003 Washington case you never seem to find any. Which way is it today Sadhusker are you importing it or not. If you are doing the proper testing then why aren't you finding it?

You tell us the formula/clues Sadhusker. Is it when you're not taking them to court that you believe USDA or is that just a time-out until the R-Klanners get whipped into a frenzy over another roll-over auction so you can gather funds to take another run at those imports.

 

[Sandhusker]

Yes, we are importing it, Bill. Can you refute that?

 

[don]

maybe you aren't importing it if you aren't finding it. if usda's number of positive american cows is good enough for you then the number of canadian positives found in the states should also be valid. as well, if you are importing it why isn't it being propagated through your non feedban? you also have the distinction of having atypical (aren't you special?) bse so if you find any regular old common bse it gives you deniability but that card hasn't even been played yet. what the heck is going on in the old divided states of america? you must have crap for a testing program.

 

[flounder]

snip...

you also have the distinction of having atypical (aren't you special?) bse so if you find any regular old common bse it gives you deniability but that card hasn't even been played yet. what the heck is going on in the old divided states of america? you must have crap for a testing program.

Where has atypical BSE been found?

Although the greatest number of cases is in France(12), increasing numbers of cases
have now been identified in other countries – Canada (1), Germany (2), Italy (2),
Japan (2), Netherlands (4), Poland (7), Sweden (1), Switzerland (1), UK (1), and USA
(2). In Sweden and the USA the atypical cases represent the only indigenous cases
detected. In other words – typical BSE has not been detected in native cattle in these
two countries (34).
In France, Poland, Netherlands and Germany both H and L forms of atypical BSE
have been reported(25).


http://www.tafsforum.org/position_papers/TAFS_POSITION_PAPER_ON_ATYPICAL_SCRAPIE_AND_%20ATYPICAL_BSE_070516.pdf



> It was, however, performed in the USA in 1979, when it was shown that
cattle
> inoculated with the scrapie agent endemic in the flock of Suffolk sheep at
> the United States Department of Agriculture in Mission, Texas, developed a
> TSE quite unlike BSE. 32 The findings of the initial transmission, though
> not of the clinical or neurohistological examination, were communicated in
> October 1988 to Dr Watson, Director of the CVL, following a visit by Dr
> Wrathall, one of the project leaders in the Pathology Department of the
CVL,
> to the United States Department of Agriculture. 33 The results were not
> published at this point, since the attempted transmission to mice from the
> experimental cow brain had been inconclusive. The results of the clinical
> and histological differences between scrapie-affected sheep and cattle
were
> published in 1995. Similar studies in which cattle were inoculated
> intracerebrally with scrapie inocula derived from a number of
> scrapie-affected sheep of different breeds and from different States, were
> carried out at the US National Animal Disease Centre. 34 The results,
> published in 1994, showed that this source of scrapie agent, though
> pathogenic for cattle, did not produce the same clinical signs of brain
> lesions characteristic of BSE.
>
> http://www.bseinquiry.gov.uk/
>
>
>
> 1: J Infect Dis. 1994 Apr;169(4):814-20.
>
>
> Intracerebral transmission of scrapie to cattle.
>
> Cutlip RC, Miller JM, Race RE, Jenny AL, Katz JB, Lehmkuhl HD, DeBey BM,
> Robinson MM.
>
> USDA, Agriculture Research Service, National Animal Disease Center, Ames,
IA
> 50010.
>
> To determine if sheep scrapie agent(s) in the United States would induce a
> disease in cattle resembling bovine spongiform encephalopathy, 18 newborn
> calves were inoculated intracerebrally with a pooled suspension of brain
> from 9 sheep with scrapie. Half of the calves were euthanatized 1 year
after
> inoculation. All calves kept longer than 1 year became severely lethargic
> and demonstrated clinical signs of motor neuron dysfunction that were
> manifest as progressive stiffness, posterior paresis, general weakness,
and
> permanent recumbency. The incubation period was 14-18 months, and the
> clinical course was 1-5 months. The brain from each calf was examined for
> lesions and for protease-resistant prion protein. Lesions were subtle, but
a
> disease-specific isoform of the prion protein was present in the brain of
> all calves. Neither signs nor lesions were characteristic of those for
> bovine spongiform encephalopathy.
>
> MeSH Terms:
> Animals
> Brain/microbiology*
> Brain/pathology
> Cattle
> Cattle Diseases/etiology*
> Cattle Diseases/pathology
> Encephalopathy, Bovine Spongiform/etiology*
> Encephalopathy, Bovine Spongiform/pathology
> Immunoblotting/veterinary
> Immunohistochemistry
> Male
> Motor Neurons/physiology
> Prions/analysis
> Scrapie/pathology
> Scrapie/transmission*
> Sheep
> Sleep Stages
> Time Factors
>
> Substances:
> Prions
>
>
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_ui
> ds=8133096&dopt=Citation
>
>
> Intracerebral transmission of scrapie to cattle FULL TEXT PDF;
>
> SNIP...
>
>
> Discussion
>
>
> WE conclude that American sources of sheep scrapie are transmissible to
> cattle by direct intracerebral inoculation but the disease induced is NOT
> identical to BSE as seen in the United Kingdom. While there were
> similarities in clinical signs between this experimental disease and BSE,
> there was no evidence of aggressiveness, hyperexcitability, hyperesthesia
> (tactile or auditory), or hyperemetria of limbs as has been reported for
BSE
> (9). Neither were there extensive neurologic lesions, which are primary
for
> BSE, such as severe vacuolation of neurons and neuropil or neuronal
necrosis
> and gliosis. Although some vacuolation of neuropil, chromotolysis in
> neurons, and gliosis were seen in the brains of some affected calves,
these
> were industinguishable from those of controls. Vacuolated neurons in the
red
> nucleus of both challenged and normal calves were considered normal for
the
> bovines as previously described (50).
>
>
> PrP-res was found in ALL CHALLENGED CALVES REGARDLESS OF CLINCIAL SIGNS,
and
> the amount of PrP-res positively related to the length of the incubation.
> ...
>
>
> snip...
>
>
> WE also conclude from these studies that scrapie in cattle MIGHT NOT BE
> RECOGNIZED BY ROUTINE HISTOPATHOLOGICAL EXAMINATION OF THE BRAIN AND
SUGGEST
> THAT DETECTION OF PrP-res by immunohistochemistry or immunoblotting is
> necessary to make a definitive diagnosis. THUS, undiagnosed scrapie
> infection could contribute to the ''DOWNER-COW'' syndrome and could be
> responsible for some outbreaks of transmissible mink encephalopathy
proposed
> by Burger and Hartsough (8) and Marsh and harsough (52). ...
>
>
> snip...
>
>
> Multiple sources of sheep affected with scrapie and two breeds of cattle
> from several sources were used inthe current study in an effort to avoid a
> single strain of either agent or host. Preliminary results from mouse
> inoculations indicate multiple strains of the agent were present in the
> pooled inoculum (unpublished data). ...
>
>
> Transmission of the sheep scrapie to cattle was attempted in 1979 by using
> intracerebral, intramuscular, subcutaneous, and oral routes of inoculation
> of 5, 8- to 11-month old cattlw with a homologous mixture of brain from 1
> affected sheep (61, 62). ONE of the 5 cattle develped neurologic signs 48
> months after inoculation. Signs were disorientation, incoordination, a
> stiff-legged stilted gait, progressive difficulty in rising, and finally
in
> terminal recumbency. The clinical course was 2.5 months. TWO of the 5
cattle
> similarly inoculated with brain tissue from a goat with scrapie exhibited
> similar signs 27 and 36 months after incoluation. Clinical courses were 43
> an 44 days. Brain lesions of mild gliosis and vacuolation and mouse
> inoculation data were insufficient to confirm a diagnosis of scrapie. This
> work remained controversial until recent examination of the brains
detected
> PrP-res in all 3 cattle with neurologic disease but in none of the
> unaffected cattle (62). Results of these studies are similar to ours and
> underscore the necessity of methods other than histopathology to diagnose
> scrapie infection in cattle. We believe that immunologic techniques for
> detecting PrP-res currently provide the most sensitive and reliable way to
> make a definitive diagnosis...
>
>
> http://www.bseinquiry.gov.uk/files/sc/seac17/tab03.pdf
>
>
> Visit to USA ... info on BSE and Scrapie
>
> http://www.bseinquiry.gov.uk/files/yb/1988/10/00001001.pdf
>



ANIMAL HEALTH REPORT 2006 (BSE h-BASE EVENT IN ALABAMA, Scrapie, and CWD)

http://animalhealthreport2006.blogspot.com/


CREUTZFELDT JAKOB DISEASE MAD COW BASE UPDATE USA

http://cjdmadcowbaseoct2007.blogspot.com/


BSE BASE MAD COW TESTING TEXAS, USA, AND CANADA

http://madcowtesting.blogspot.com/


NOR-98 ATYPICAL SCRAPIE USA UPDATE AS AT OCT 2007

http://nor-98.blogspot.com/

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


tss

 

[Sandhusker]

don, can you show me where the USDA is trying to find any imported BSE?

 

[don]

no because if they ran the kind of testing program finding imported they might stumble onto more native that they couldn't avoid.

 

[Bill]

Yes, we are importing it, Bill. Can you refute that?

Refute that? Refute WHAT? Where are all the cases you seem to think are coming across the border into the US. You have found ONE; count 'em ONE imported case

Where is your proof that you are importing it. Why would I have to refute something you can't find.

Maybe the girls at the bank can explain it to you tomorrow.

 

[Sandhusker]

no because if they ran the kind of testing program finding imported they might stumble onto more native that they couldn't avoid.

Since you're aware that they're not looking for it, how can you make any conclusions based on them not finding any?

 

[Sandhusker]

Yes, we are importing it, Bill. Can you refute that?

Refute that? Refute WHAT? Where are all the cases you seem to think are coming across the border into the US. You have found ONE; count 'em ONE imported case

Where is your proof that you are importing it. Why would I have to refute something you can't find.

Maybe the girls at the bank can explain it to you tomorrow.

Refute that BSE positive animals are not coming across the border. Tell me how you're identifying and then stopping them from coming down - OR - tell me how you don't have BSE up there.

 

[Kathy]

Genetically modified mice and intracranial injections with homogenized brain tissue..... AGAIN.... So many cheap cows available to work with, and they keep using GM mice.... 25 years have passed by, their excuse that it takes to long to develop the disease in cattle just doesn't cut it.

A well respected prion scientist from Switzerland made a comment in this abstract that is quite intriging also:

Annu Rev Pathol. 2008;3:11-40.
Molecular mechanisms of prion pathogenesis.

Aguzzi A, Sigurdson C, Heikenwaelder M.
Institute of Neuropathology, University Hospital of Zürich, CH-8091 Zürich, Switzerland; email: [email protected]. , [email protected]. , [email protected]..

Prion diseases are infectious neurodegenerative diseases occurring in humans and animals with an invariably lethal outcome. One fundamental mechanistic event in prion diseases is the aggregation of aberrantly folded prion protein into large amyloid plaques and fibrous structures associated with neurodegeneration. The cellular prion protein (PrP(C)) is absolutely required for disease development, and prion knockout mice are not susceptible to prion disease. Prions accumulate not only in the central nervous system but also in lymphoid organs, as shown for new variant and sporadic Creutzfeldt-Jakob patients and for some animals. To date it is largely accepted that prions consist primarily of PrP(Sc), a misfolded and aggregated beta-sheet-rich isoform of PrP(C). However, PrP(Sc) may or may not be completely congruent with the infectious moiety. Here, we discuss the molecular mechanisms leading to neurodegeneration, the role of the immune system in prion pathogenesis, and the existence of prion strains that appear to have different tropisms and biochemical characteristics.

PMID: 18233951

The cellular prion protein is absolutely required for life. Even the PrP(C) free cloned animals of spefic labs have required the existence of PrPC in the cells to create new viable organisms. Only after a certain stage of "development" were they able to "shut off" the genes of PrPC. Even then, the creatures suffered from excessive oxidative stress which results in premature aging and death.... just like the sheep Dolly.

Only "aggregates" and "fibrils" are "associated with" neurodegeneration.

It is kind of them to finally acknowledge that the PrP(Sc) protein MAY NOT BE the infectious agent.

This fits with Dr. Vodyanoy's hypothesis that the protein nucleating centers (PNCs) consisting of metal nanoclusters with 40-300 atoms are the core problem. Their energy allows for the misfolding, polymerization and aggregation of the various proteins.

If you care to read an interesting paper, available on-line free, here is the link. The paper is at:

http://www.pubmedcentral.nih.gov/articlerender.fcgi?tool=pubmed&pubmedid=17373858

"Protein Oxidation Implicated as the primary determinant of Bacterial Radioresistance" Michael Daly et al. April 2007 PLoS Biology Volume 5 Issue 4.

In the hierarchy of cellular targets damaged by ionizing radiation (IR), classical models of radiation toxicity place DNA at the top. Yet, many prokaryotes are killed by doses of IR that cause little DNA damage. Here we have probed the nature of Mn-facilitated IR resistance in Deinococcus radiodurans, which together with other extremely IR-resistant bacteria have high intracellular Mn/Fe concentration ratios compared to IR-sensitive bacteria. For in vitro and in vivo irradiation, we demonstrate a mechanistic link between Mn(II) ions and protection of proteins from oxidative modifications that introduce carbonyl groups. Conditions that inhibited Mn accumulation or Mn redox cycling rendered D. radiodurans radiation sensitive and highly susceptible to protein oxidation. X-ray fluorescence microprobe analysis showed that Mn is globally distributed in D. radiodurans, but Fe is sequestered in a region between dividing cells. For a group of phylogenetically diverse IR-resistant and IR-sensitive wild-type bacteria, our findings support the idea that the degree of resistance is determined by the level of oxidative protein damage caused during irradiation. We present the case that protein, rather than DNA, is the principal target of the biological action of IR in sensitive bacteria, and extreme resistance in Mn-accumulating bacteria is based on protein protection.

PMID: 17373858

These researchers have shown how ionizing radiation induced an increase in manganese in the bacteria resistant to the damage caused by the radiation. They demonstrate how iron from the bacteria became "sequestered in a region between dividing cells". Very interesting, and similar to prion propagation.

Taken into consideration with other studies showing how manganese (especially manganese based SODII) is upregulated with radiation exposure, as well as elevated manganese in the brain tissue of CJD victims;

Mol Cell Neurosci. 2007 Dec 15 [Epub ahead of print]

Elevated manganese levels in blood and CNS in human prion disease.
Hesketh S, Sassoon J, Knight R, Brown DR.
Department of Biology and Biochemistry, University of Bath, Bath, BA2 7AY, UK.

Prion disease or transmissible spongiform encephalopathies are neurodegenerative disorders of humans and other mammals. They are fatal and difficult to diagnose. Previous studies have suggested that some prion diseases cause elevation of manganese in the blood and brain. In the current study we analysed blood and brain samples from humans to determine whether elevation in manganese is a specific characteristic of Creutzfeldt-Jakob disease, the most common form of human prion disease. Analysis of manganese in the blood of normal humans showed that concentrations vary little with age or sex. Analysis of other diseases, including other neurodegenerative disease showed that only CJD showed an elevation in manganese and copper. Other diseases that showed elevated manganese included blood-brain barrier disorders and haemochromatosis. However, CJD could be easily distinguished from these diseases. This implies that increased blood manganese in prion disease is a highly specific characteristic of the disease.

PMID: 18234506

This new paper from D.R. Brown of Bath, UK, continues the investigation looking for presymptomatic analysis of prion diseases. The elevated manganese (and even copper at first) demonstrates that the body is fighting against an attach. A chronic exposure to the agent results in wearing down these defense mechanisms. The prion protein, (being normally a copper loaded protein), when depleted of copper is contaminated with manganese and other metals. The lack of function results in increased oxidative stress within the cell and body..... protein and lipid peroxidation....

Elevated manganese is a huge clue in the puzzle..... review of the Protein Oxidation paper above, will help to disclose how manganese protects certain radioresistant bacteria from damage by ionizing radiation. Humans have not evolved to have this "radioresistance" as some bacteria have. Chernobyl fallout, DU, nuclear reactors, radio-active contamination of the environment (natural and man-made) and mineral excesses and deficiencies have lead us to all this mess. Following the hyper-infectious protein only hypothesis, will only lead us deeper into the quagmire.