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From: TSS ()
Subject: CJD AND FARMERS AND BaSEcjd
Date: March 23, 2005 at 2:45 pm PST

-------- Original Message --------
Subject: CJD AND FARMERS AND BaSEcjd
Date: Wed, 23 Mar 2005 16:34:56 -0600
From: "Terry S. Singeltary Sr."
Reply-To: Bovine Spongiform Encephalopathy
To: BSE-L@KALIV.UNI-KARLSRUHE.DE


##################### Bovine Spongiform Encephalopathy #####################

MAYBE these farmers had BaSEcjd ???

provides information on a probable case of CJD in a farmer,
if confirmed, would bring the total number of cases in farmers
in the UK to four-all with BSE in their herds...

http://www.bseinquiry.gov.uk/files/yb/1995/09/29001001.pdf
http://www.bseinquiry.gov.uk/files/yb/1995/09/29008001.pdf
http://www.bseinquiry.gov.uk/files/yb/1995/09/29009001.pdf
http://www.bseinquiry.gov.uk/files/yb/1995/09/29012001.pdf
http://www.bseinquiry.gov.uk/files/yb/1995/09/29013001.pdf
http://www.bseinquiry.gov.uk/files/yb/1995/10/04003001.pdf
http://www.bseinquiry.gov.uk/files/yb/1995/10/04004001.pdf
http://www.bseinquiry.gov.uk/files/yb/1995/10/04005001.pdf
http://www.bseinquiry.gov.uk/files/yb/1995/10/04006001.pdf
http://www.bseinquiry.gov.uk/files/yb/1995/10/05004001.pdf
http://www.bseinquiry.gov.uk/files/yb/1995/10/10004001.pdf
http://www.bseinquiry.gov.uk/files/yb/1995/10/19002001.pdf

CAUSE FOR CONCERN IN 3RD FARMER WITH CJD

http://www.bseinquiry.gov.uk/files/yb/1995/01/13001001.pdf
http://www.bseinquiry.gov.uk/files/yb/1995/01/19002001.pdf

6. Members were reminded that, after the death from CJD of the
second dairy farmer in 1993, Professor Peter Smith had advised that
IF FOUR CASES AROSE in the first five years of the surveillance
scheme the possibility of an association which was NOT DUE TO
CHANCE HAD TO BE GIVEN VERY SERIOUS CONSIDERATION...

http://www.bseinquiry.gov.uk/files/yb/1995/01/13001001.pdf

cover-up 4th farmer from cjd

http://www.bseinquiry.gov.uk/files/yb/1995/10/23006001.pdf

http://www.bseinquiry.gov.uk/files/yb/1995/10/23007001.pdf

Government experts hold secret inquiry as fourth farmer with
infected cattle falls victim to deadly brain disease

http://www.bseinquiry.gov.uk/files/yb/1995/10/23010001.pdf

FOURTH FARMER CONFIRMED
should there be any further press enquiries specifically about the diagnosis
in this case, I therfore suggest that we say that the diagnosis has been
confimred but that there is nothing to add to SEACs earlier advice.
http://www.bseinquiry.gov.uk/files/yb/1995/11/03008001.pdf

IT WAS ALSO AGREED THAT IF A THIRD CASE OF CJD IN
A FARMER WITH BSE IN THEIR HER OCCURED, AN IMMEDIATE
FULL COMMITTEE MEETING WOULD BE REQUIRED...

http://www.bseinquiry.gov.uk/files/yb/1993/10/07002001.pdf

10. Dr. Will presented information on CJD in farmers in other
European countries - three cases in France in 1992 and 1993,
two of which were dairy farmers, and two cases in dairy farmers
in Germany since October 1993.

http://www.bseinquiry.gov.uk/files/yb/1995/01/13001001.pdf

IF this new strain of TSE in cattle aBSE or BaSE is in older cattle.
IF this new strain of TSE in cattle looks IDENTICAL to sporadic CJD.
WHY is it so hard to believe that these sporadic CJD deaths in farmers
we not related to this Base or BaSE?...TSS


Medical Sciences
Identification of a second bovine amyloidotic spongiform
encephalopathy: Molecular similarities with sporadic
Creutzfeldt-Jakob disease

Cristina Casalone *{dagger} , Gianluigi Zanusso {dagger} {ddagger} ,
Pierluigi Acutis *, Sergio Ferrari {ddagger} , Lorenzo Capucci § ,
Fabrizio Tagliavini ¶, Salvatore Monaco {ddagger} ||, and Maria Caramelli *

*Centro di Referenza Nazionale per le Encefalopatie Animali, Istituto
Zooprofilattico Sperimentale del Piemonte, Liguria e Valle d'Aosta, Via
Bologna, 148, 10195 Turin, Italy; {ddagger} Department of Neurological
and Visual Science, Section of Clinical Neurology, Policlinico G.B.
Rossi, Piazzale L.A. Scuro, 10, 37134 Verona, Italy; § Istituto
Zooprofilattico Sperimentale della Lombardia ed Emilia Romagna, Via
Bianchi, 9, 25124 Brescia, Italy; and ¶Istituto Nazionale Neurologico
"Carlo Besta," Via Celoria 11, 20133 Milan, Italy

Edited by Stanley B. Prusiner, University of California, San Francisco,
CA, and approved December 23, 2003 (received for review September 9, 2003)

Transmissible spongiform encephalopathies (TSEs), or prion diseases, are
mammalian neurodegenerative disorders characterized by a
posttranslational conversion and brain accumulation of an insoluble,
protease-resistant isoform (PrPSc) of the host-encoded cellular prion
protein (PrPC). Human and animal TSE agents exist as different
phenotypes that can be biochemically differentiated on the basis of the
molecular mass of the protease-resistant PrPSc fragments and the degree
of glycosylation. Epidemiological, molecular, and transmission studies
strongly suggest that the single strain of agent responsible for bovine
spongiform encephalopathy (BSE) has infected humans, causing variant
Creutzfeldt-Jakob disease. The unprecedented biological properties of
the BSE agent, which circumvents the so-called "species barrier" between
cattle and humans and adapts to different mammalian species, has raised
considerable concern for human health. To date, it is unknown whether
more than one strain might be responsible for cattle TSE or whether the
BSE agent undergoes phenotypic variation after natural transmission.
Here we provide evidence of a second cattle TSE. The disorder was
pathologically characterized by the presence of PrP-immunopositive
amyloid plaques, as opposed to the lack of amyloid deposition in typical
BSE cases, and by a different pattern of regional distribution and
topology of brain PrPSc accumulation. In addition, Western blot analysis
showed a PrPSc type with predominance of the low molecular mass
glycoform and a protease-resistant fragment of lower molecular mass than
BSE-PrPSc. Strikingly, the molecular signature of this previously
undescribed bovine PrPSc was similar to that encountered in a distinct
subtype of sporadic Creutzfeldt-Jakob disease.

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

{dagger} C.C. and G.Z. contributed equally to this work.

||To whom correspondence should be addressed.

E-mail: salvatore.monaco@mail.univr.it
.
www.pnas.org/cgi/doi/10.1073/pnas.0305777101

http://www.pnas.org/cgi/content/abstract/0305777101v1

BSE prions propagate as either variant CJD-like or sporadic CJD-like
prion strains in transgenic mice expressing human prion protein

Emmanuel A. Asante, Jacqueline M. Linehan, Melanie Desbruslais, Susan
Joiner, Ian Gowland, Andrew L. Wood, Julie Welch, Andrew F. Hill, Sarah
E. Lloyd, Jonathan D.F. Wadsworth and John Collinge1

MRC Prion Unit and Department of Neurodegenerative Disease, Institute of
Neurology, University College, Queen Square, London WC1N 3BG, UK 1
Corresponding author e-mail: j.collinge@prion.ucl.ac.uk


Received August 1, 2002; revised September 24, 2002; accepted October
17, 2002

Abstract


Variant CreutzfeldtJakob disease (vCJD) has been recognized to date
only in individuals homozygous for methionine at PRNP codon 129. Here we
show that transgenic mice expressing human PrP methionine 129,
inoculated with either bovine spongiform encephalopathy (BSE) or variant
CJD prions, may develop the neuropathological and molecular phenotype of
vCJD, consistent with these diseases being caused by the same prion
strain. Surprisingly, however, BSE transmission to these transgenic
mice, in addition to producing a vCJD-like phenotype, can also result in
a distinct molecular phenotype that is indistinguishable from that of
sporadic CJD with PrPSc type 2. These data suggest that more than one
BSE-derived prion strain might infect humans; it is therefore possible
that some patients with a phenotype consistent with sporadic CJD may
have a disease arising from BSE exposure...

http://embojournal.npgjournals.com/cgi/content/full/21/23/6358


S. E. Lloyd, J. M. Linehan, M. Desbruslais, S. Joiner, J. Buckell, S.
Brandner, J. D. F. Wadsworth, and J. Collinge
Characterization of two distinct prion strains derived from bovine
spongiform encephalopathy transmissions to inbred mice
J. Gen. Virol., August 1, 2004; 85(8): 2471 - 2478.
[Abstract]
[Full Text]
[PDF]

Adaptation of the bovine spongiform encephalopathy agent to primates
and comparison with Creutzfeldt- Jakob disease: Implications for
human health

THE findings from Corinne Ida Lasmézas*, [dagger] , Jean-Guy Fournier*,
Virginie Nouvel*,

Hermann Boe*, Domíníque Marcé*, François Lamoury*, Nicolas Kopp [Dagger

] , Jean-Jacques Hauw§, James Ironside¶, Moira Bruce [||] , Dominique

Dormont*, and Jean-Philippe Deslys* et al, that The agent responsible
for French iatrogenic growth hormone-linked CJD taken as a control is
very different from vCJD but is similar to that found in one case of
sporadic CJD and one sheep scrapie isolate;

http://www.pnas.org/cgi/content/full/041490898v1

TSS

######### https://listserv.kaliv.uni-karlsruhe.de/warc/bse-l.html ##########





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