TimH wrote:
[/quote]
Well Porky, Hundreds of millions of people in the Uk and North America, consume millions of pounds of dairy products daily.....and have done so since long before BSE was identified(20+ years ago). What's the tally on worldwide cases of vCJD to date???? 160 give or take????
I"ll take my chances and drink some milk and eat some beef.
Scare somebody else in to buying "scoring ag".
[/quote]
more to the story than just the nvCJD strain in humans, and just the BSE strain in cattle.
more to the story than oral consumption.
Monday, November 5, 2007
PRO/AH/EDR> Prion disease update 2007 (07)
PRION DISEASE UPDATE 2007 (07)
******************************
A ProMED-mail post
<http://www.promedmail.org>
ProMED-mail is a program of the
International Society for Infectious Diseases
<http://www.isid.org>
snip...
******
[2] USA: National Prion Disease Pathology Surveillance Center
Date: June 2007
Source: National Prion Disease Pathology Surveillance Center (USA) [edited]
<http://www.cjdsurveillance.com/pdf/case-table.pdf>
CJD Cases examined
----------------------
Year / Referrals / Prion disease / Sporadic / Familial / Iatrogenic / vCJD
1996 / 42 / 32 / 26 / 4 / 0 / 0
1997 / 115 / 68 / 57 / 9 / 0 / 0
1998 / 93 / 53 / 45 / 7 / 1 / 0
1999 / 114 / 69 / 61 / 8 / 0 / 0
2000 / 151 / 103 / 89 / 14 / 0 / 0
2001 / 208 / 116 / 106 / 9 / 0 / 0
2002 / 255 / 143 / 118 / 23 / 2 / 0
2003 / 272 / 174 / 132 / 41 / 0 / 0
2004 / 334 / 183 / 157 / 21 / 0 / 1*
2005 / 352 / 195 / 152 / 37 / 1 / 0
2006 / 372 / 186 / 143 / 30 / 0 / 1**
2007 / 120 / 68 / 35 / 7 / 0 / 0
TOTAL / 2428*** / 1390**** / 1121 / 210 / 4 / 2
*Acquired in UK
** Acquired in Saudi Arabia
*** Includes 17 inconclusive and 9 pending (1 from 2006, 8 from 2007.
**** Includes 17 non-vCJD type unknown (2 from 1996, 2 from 1997, 1
from 2001, 1 from 2003, 4 from 2004, 3 from 2005, 4 from 2006) and 36
type pending (2 from 2005, 8 from 2006, 26 from 2007).
Notes:
-- Cases are listed based on the year of death when available. If the
year of death is not available, the year of sample receipt is used.
-- Referrals: Cases with possible or probable prion disease from
which brain tissue or blood in the case of familial disease were submitted.
-- Inconclusive: Cases in which the samples were not sufficient to
make a diagnosis.
-- Non-vCJD type unknown are cases in which the tissue submitted was
adequate to establish the presence but not the type; in all cases,
vCJD could be excluded.
--
Communicated by:
Terry S. Singeltary Sr. <
[email protected]>
[In submitting these data, Terry S. Singeltary Sr. draws attention to
the steady increase in the "type unknown" category, which, according
to their definition, comprises cases in which vCJD could be excluded.
The total of 26 cases for the current year (2007) is disturbing,
possibly symptomatic of the circulation of novel agents.
Characterization of these agents should be given a high priority. - Mod.CP]
*##########################################################*
http://pro-med.blogspot.com/2007/11/proahedr-prion-disease-update-2007-07.html
FC5.5.1
BASE Transmitted to Primates and MV2 sCJD Subtype Share PrP27-30 and PrPSc
C-terminal Truncated Fragments
Zanusso, G1; Commoy, E2; Fasoli, E3; Fiorini, M3; Lescoutra, N4; Ruchoux,
MM4; Casalone, C5; Caramelli, M5; Ferrari, S3; Lasmezas, C6; Deslys, J-P4;
Monaco, S3 1University of Verona, of Neurological and Visual Sciences,
Italy; 2CEA, IMETI/SEPIA, France; 3University of Verona, Neurological and
Visual Sciences, Italy; 4IMETI/SEPIA, France; 5IZSPLVA, Italy; 6The Scripps
Research Insitute, USA
The etiology of sporadic Creutzfeldt-Jakob disease (sCJD), the most frequent human
prion disease, remains still unknown. The marked disease phenotype heterogeneity
observed in sCJD is thought to be influenced by the type of proteinase K-resistant
prion protein, or PrPSc (type 1 or type 2 according to the electrophoretic mobility of
the unglycosylated backbone), and by the host polymorphic Methionine/Valine (M/V)
codon 129 of the PRNP. By using a two-dimensional gel electrophoresis (2D-PAGE)
and imunoblotting we previously showed that in sCJD, in addition to the PrPSc type,
distinct PrPSc C-terminal truncated fragments (CTFs) correlated with different sCJD
subtypes. Based on the combination of CTFs and PrPSc type, we distinguished three
PrPSc patterns: (i) the first was observed in sCJD with PrPSc type 1 of all genotypes,;
(ii) the second was found in M/M-2 (cortical form); (iii) the third in amyloidogenic M/V-
2 and V/V-2 subtypes (Zanusso et al., JBC 2004) . Recently, we showed that sCJD
subtype M/V-2 shared molecular and pathological features with an atypical form of
BSE, named BASE, thus suggesting a potential link between the two conditions. This
connection was further confirmed after 2D-PAGE analysis, which showed an
identical PrPSc signature, including the biochemical pattern of CTFs. To pursue this
issue, we obtained brain homogenates from Cynomolgus macaques intracerebrally
inoculated with brain homogenates from BASE. Samples were separated by using
a two-dimensional electrophoresis (2D-PAGE) followed by immunoblotting. We here
show that the PrPSc pattern obtained in infected primates is identical to BASE
and sCJD MV-2 subtype. These data strongly support the link, or at least a common
ancestry, between a sCJD subtype and BASE. This work was supported by Neuroprion
(FOOD-CT-2004-506579)
FC5.5.2 Transmission of Italian BSE and BASE Isolates in Cattle Results into a
Typical BSE Phenotype and a Muscle Wasting Disease
Zanusso, G1; Lombardi, G2; Casalone, C3; D'Angelo, A4; Gelmetti, D2; Torcoli, G2;
Barbieri, I2; Corona, C3; Fasoli, E1; Farinazzo, A1; Fiorini, M1; Gelati, M1; Iulini, B3;
Tagliavini, F5; Ferrari, S1; Monaco, S1; Caramelli, M3; Capucci, L2
1University of Verona, Neurological and Visual Sciences, Italy; 2IZSLER,
Italy; 3IZSPLVA, Italy; 4University of Turin, Animal Pathology, Italy; 5Isituto Carlo Besta,
Italy
The clinical phenotype of bovine spongiform encephalopathy has been extensively
reported in early accounts of the disorder. Following the introduction of statutory active
surveillance, almost all BSE cases have been diagnosed on a pathological/molecular
basis, in a pre-symptomatic clinical stage. In recent years, the active surveillance
system has uncovered atypical BSE cases, which are characterized by distinct
conformers of the PrPSc, named high-type (BSE-H) and low-type (BSE-L), whose
clinicopathological phenotypes remain unknown. We recently reported two
Italian atypical cases with a PrPSc type similar to BSE-L, pathologically
characterized by PrP amyloid plaques. Experimental transmission to TgBov
mice has recently disclosed that BASE is caused by a distinct prion strain which is
extremely virulent. A major limitation of transmission studies to mice is the lack of reliable
information on clinical phenotype of BASE in its natural host. In the present study, we
experimentally infected Fresian/Holstein and Alpine/Brown cattle with Italian BSE and
BASE isolates by i.c. route. BASE infected cattle showed survival times significantly
shorter than BSE, a finding more readily evident in Fresian/Holstein, and in keeping with
previous observations in TgBov mice. Clinically, BSE-infected cattle developed a
disease phenotype highly comparable with that described in field BSE cases
and in experimentally challenged cattle. On the contrary, BASE-inoculated cattle
developed an amyotrophic disorder accompanied by mental dullness.
The molecular and neuropathological profiles, including PrP deposition
pattern, closely matched those observed in the original cases. This study further confirms
that BASE is caused by a distinct prion isolate and discloses a novel disease
phenotype in cattle, closely resembling the phenotype previous reported in scrapie-inoculated
cattle and in some subtypes of inherited and sporadic Creutzfeldt-Jakob disease.
Oral Abstracts
14
P01.34
Pathological Interaction Between Protein Misfolding Disorders: Prions and
Alzheimer's Disease
Morales, R; Estrada, L; Castilla, J; Soto, C
University of Texas Medical Branch, Neurology, USA
Protein Misfolding Disorders (PMD) include several diverse diseases, such Alzheimer's,
Parkinson's, Transmissible Spongiform Encephalopathies, Diabetes Type II and various
systemic amyloidosis. The central event in these diseases is the accumulation of a
misfolded, ß-sheet rich aggregated form of a naturally expressed protein. In vitro
studies have shown that protein misfolding and aggregation follows a seedingnucleation
mechanism similar to the process of crystallization. In this model, the
limiting step is the formation of small oligomeric intermediates that act as seeds to
catalyze the polymerization process. The seeding-nucleation model provides a
rationale and plausible explanation for the infectious nature of prions. Infectivity lies on
the capacity of preformed stable misfolded oligomeric proteins to act as a seed to
catalyze the misfolding and aggregation process. The mechanism of misfolding and
aggregation is similar in all PMD suggesting that misfolded aggregates have an
inherent capability to be transmissible. Moreover, it has been shown that oligomeric
seeds formed by one protein can accelerate the misfolding and aggregation of another
protein, by a process termed cross-seeding. Our current study aims to assess the
potential molecular cross-talk among PMD in vivo. For this purpose we inoculated with
prions a transgenic mice model of Alzheimer's disease (tg2576) that develops typical
amyloid plaques over time. 45, 303 and 365 days old transgenic and wild type mice
were inoculated intraperitoneally with RML prions. We found significant diminution in
prion incubation periods for tg2576 mice compared to age matched wild type controls.
Moreover, a time dependent effect was observed, where the shorter incubation period
was observed in animals containing larger number of amyloid plaques. Inoculation of
tg2576-RML prions into wild type mice showed incubation periods similar to the
original infectious material, suggesting that strains characteristics are maintained. In
vitro data showed cross-seeding aggregation between PrPSc and Aß. Our findings
suggest an interaction between Alzheimer's and prion pathologies, indicating that one
protein misfolding process may be an important risk factor for the development of a
second perhaps more prevalent disease.
see full text 143 pages ;
http://www.prion2007.com/pdf/Prion%20Book%20of%20Abstracts.pdf
18 January 2007 - Draft minutes of the SEAC 95 meeting (426 KB) held on 7
December 2006 are now available.
snip...
64. A member noted that at the recent Neuroprion meeting, a study was
presented showing that in transgenic mice BSE passaged in sheep may be more
virulent and infectious to a wider range of species than bovine derived BSE.
***
Other work presented suggested that BSE and bovine amyloidotic spongiform
encephalopathy (BASE) MAY BE RELATED. A mutation had been identified in the
prion protein gene in an AMERICAN BASE CASE THAT WAS SIMILAR IN NATURE TO A
MUTATION FOUND IN CASES OF SPORADIC CJD.
***
snip...
http://www.seac.gov.uk/minutes/95.pdf
3:30 Transmission of the Italian Atypical BSE (BASE) in Humanized Mouse
Models Qingzhong Kong, Ph.D., Assistant Professor, Pathology, Case Western
Reserve
University
Bovine Amyloid Spongiform Encephalopathy (BASE) is an atypical BSE strain
discovered recently in Italy, and similar or different atypical BSE cases
were also reported in other countries. The infectivity and phenotypes of
these atypical BSE strains in humans are unknown. In collaboration with
Pierluigi Gambetti, as well as Maria Caramelli and her co-workers, we have
inoculated transgenic mice expressing human prion protein with brain
homogenates from BASE or BSE infected cattle. Our data shows that about half
of the BASE-inoculated mice became infected with an average incubation time
of about 19 months; in contrast, none of the BSE-inoculated mice appear to
be infected after more than 2 years.
***
These results indicate that BASE is transmissible to humans and suggest
that BASE is more virulent than classical BSE in humans.
***
6:30 Close of Day One
http://www.healthtech.com/2007/tse/day1.asp
***
There is a growing number of human CJD cases, and they were presented last
week in San Francisco by Luigi Gambatti(?) from his CJD surveillance
collection.
***
He estimates that it may be up to 14 or 15 persons which display selectively
SPRPSC and practically no detected RPRPSC proteins.
***
http://www.fda.gov/ohrms/dockets/ac/06/transcripts/1006-4240t1.htm
http://www.fda.gov/ohrms/dockets/ac/06/transcripts/2006-4240t1.pdf
***
If, on the other hand, atypical BSE continues to occur as typical BSE
disappears, this would be a strong indication that it is indeed sporadic,
and if in addition at least 1 form of what is presently considered as
sporadic CJD (such as the type 2 M/V subtype shown to have a Western blot
signature like BASE) were to increase, this would suggest (although not
prove) a causal relationship (Figure 5).
***
http://www.cdc.gov/ncidod/EID/vol12no12/06-0965.htm
The Lancet Infectious Diseases 2003; 3:463
DOI:10.1016/S1473-3099(03)00715-1
Newsdesk
Tracking spongiform encephalopathies in North America
Xavier Bosch
"My name is Terry S Singeltary Sr, and I live in Bacliff, Texas. I lost my
mom to hvCJD (Heidenhain variant CJD) and have been searching for answers
ever since. What I have found is that we have not been told the truth. CWD
in deer and elk is a small portion of a much bigger problem."
...snip
adding that, "the cases that we know about are reassuring, because they do
not suggest the appearance of a new variant of CJD in the USA or atypical
features in patients that might be exposed to CWD. However, until we
establish a system that identifies and analyses a high proportion of
suspected prion disease cases we will not know for sure". The USA should
develop a system modelled on that established in the UK, he points
out....snip...END
http://download.thelancet.com/pdfs/journals/1473-3099/PIIS1473309903007151.pdf
SEE STEADY INCREASE IN SPORADIC CJD IN THE USA FROM
1997 TO 2006. SPORADIC CJD CASES TRIPLED, with phenotype
of 'UNKNOWN' strain growing. ...
http://www.cjdsurveillance.com/resources-casereport.html
Diagnosis and Reporting of Creutzfeldt-Jakob Disease
Singeltary, Sr et al. JAMA.2001; 285: 733-734.
http://jama.ama-assn.org/http://www.neurology.org/cgi/eletters/60/2/176#535
BRITISH MEDICAL JOURNAL
BMJ
http://www.bmj.com/cgi/eletters/319/7220/1312/b#5406
BMJ
http://www.bmj.com/cgi/eletters/320/7226/8/b#6117
JOURNAL OF NEUROLOGY
MARCH 26, 2003
RE-Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob
disease in the United States
http://www.neurology.org/cgi/eletters/60/2/176#535
THE PATHOLOGICAL PROTEIN
Hardcover, 304 pages plus photos and illustrations. ISBN 0-387-95508-9
June 2003
BY Philip Yam
CHAPTER 14 LAYING ODDS
Answering critics like Terry Singeltary, who feels that the U.S. under-
counts CJD, Schonberger conceded that the current surveillance system
has errors but stated that most of the errors will be confined to the older
population.
http://www.thepathologicalprotein.com/
USDA BSE TESTING BLUNDERS (not all)
http://lists.ifas.ufl.edu/cgi-bin/wa.exe?A2=ind0702&L=sanet-mg&D=0&T=0&P=720
MAD COW FRIENDLY FIRE
4th CASE VCJD VIA BLOOD TRANSFUSION, BSE, BASE, AND SPORADIC CJD
By Terry S Singeltary
Bacliff, Texas USA Jan 24, 07
http://bloodindex.org/view_news_zone.php?id=206
SCRAPIE USA
http://scrapie-usa.blogspot.com/
NOR-98 ATYPICAL SCRAPIE CASES USA
http://nor-98.blogspot.com/
CREUTZFELDT JAKOB DISEASE MAD COW BASE UPDATE USA
http://cjdmadcowbaseoct2007.blogspot.com/
Transmissible Mink Encephalopathy TME
http://transmissible-mink-encephalopathy.blogspot.com/
CHRONIC WASTING DISEASE
http://chronic-wasting-disease.blogspot.com/
Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob disease in
the United States
http://cjdusa.blogspot.com/
CJD QUESTIONNAIRE
http://cjdquestionnaire.blogspot.com/
Terry S. Singeltary Sr.
P.O. Box 42
Bacliff, Texas USA 77518
