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From: TSS ()
Subject: Transmission of atypical BSE to Microcebus murinus, a non-human primate: Development of clinical symptoms and tissue distribution of PrPres
Date: November 30, 2008 at 2:21 pm PST

0C3.01

Transmission of atypical BSE to Microcebus murinus, a non-human primate: Development of clinical symptoms and tissue distribution of PrPres

Background: Atypical BSE cases have been observed in Europe, Japan and North America. They differ in their PrPres profiles from those found in classical BSE. These atypical cases fall into 2 types, depending on the molecular mass of the unglycosylated PrPres band observed by Western blot: the L -type (lower molecular mass than the typical BSE cases) and H-type (higher molecular mass than the typical BSE cases).

Objectives and Methods: In order to see if the atypical BSE cases were transmissible to primates, either animals (were intracerebrally inoculated with 50 ul of a 10% brain homogenates of two atypical French BSE case, a H-type (2 males and 2 females) and a L-type (2 males and 2 females).

Results: Only one of the four lemurs challenged with H-type BSE died without clinical signs after 19 months post inoculation (mpi), whereas all the 4 animals inoculated with L -type BSE died at 19 mpi (2 males) and 22 mpi (2 females). Three months before their sacrifice, they developed blindness, tremor, abnormal posture, incoordinated movements, balance loss. Symptoms got worse according to the disease progression, until severe ataxia. The brain tissue were biochemically and immunocytochemically investigated for PrPres. For the H-type, spongiform changes without PrPres accumulation were observed in the brainstem. However Western blot analysis did not allow to detect PrPres into the brain. For the L-type, severe spongiosis was evidenced into the thalamus, the striatum, the mesencephalon, and the brainstem. whereas into the cortex the spongiosis was evidenced, but the Vacuolisation was weaker. Strong deposits of PrPres were detected by western blot, PET-blot and immunocytochemistry in the CNS: dense accumulation was observed into the thalamus, the striatum, and the hippocampus whereas in the cerebral cortex, PrPres was prominently accumulated in plaques. Western blot analysis also readily confirmed the presence of protease-resistant prion protein.

Conclusions: L-type infected lemurs showed survival times considerably shorter than for classical BSE strain, indicating that the disease is caused by a very virulent distinct prion strain in a model of non human primate.

http://www.neuroprion.org/resources/pdf_docs/conferences/prion2008/abstract-book-prion2008.pdf


P7.09

Biochemical screening for identification of atypical bse in belgium, 1999-present

Authors

Alexandre DobIy: Caroline Rodeghiero, Riet Geeroms; Stephanie Durand, Jessica De Sloovere, Emanuel Yanopdenbosch, Stefan Roels,

Content

Background: Recently atypical forms of BSE have been described. Western blot analyses showed that, in comparison to the classic BSE (C-type), they are demonstrable by a higher or lower molecular weight of the unglycosylated PrPres. They Viere thus named H-type and L-type BSE (L-type is also called BASE). In addition they show a lower proportion of diglycosylated PrPres than C-type. These emerging types represent different strains of BSE. They show unique incubation periods and histological lesions. Such types have been described on different continents. Indeed they might correspond to "sporadic" forms of BSE. In 2004 we already described one L-type in Belgium.

Objective: We retrospectively analysed the bovines at least 7-year-old in the Belgian archive of BSE ­diagnosed cattle in order to determine the prevalence of the two types of atypical BSE in Belgium.

Methods: We analysed homogenates from 39 bovines of 93 months old in median (min: 84, max: 181 months). The most recent one was diagnosed in 2006. We used Western blot with a panel of anti-PrP antibodies (Ab). They detect different regions of the PrP protein, from N-terminal to C-terminal: 12B2, 9A2, Sha31. SAFB4, 94B4. Their combination is aimed at an efficient typing diagnostic. We detected bound Ab with SuperSignal West Dura (Pierce) and analysed PrPres, signals with an image-analysis software (Quantity One, Bio-Rad).

Results: The results are still under analysis. We will detail the most crucial characteristics for typing PrPres. These include 1) the apparent molecular mass of the an-, mono- and diglycosylated bands, 2) the binding affinity to the five Ab (e.g.12B2 for H-type), 3) the presence of a fourth (unglycosylated) band and 4) the glycoprofile based on the relative proportions of the visible bands.

Discussion: The emergence of atypical types of BSE is partially due to a better knowledge of prion strains and more efficient diagnostic techniques. As the area in the brain where the PrPres is deposited can differ drastically between the types, it is essential to ascertain that the sampling techniques and analyses are adapted to these new types. As these new strains seem more virulent than classic types, they represent one of the next challenges in the field of prions.

http://www.neuroprion.org/resources/pdf_docs/conferences/prion2008/abstract-book-prion2008.pdf


http://www.prion2008.com/

Tuesday, November 11, 2008

Transmission of atypical bovine prions to mice transgenic for human prion protein

DOI: 10.3201/eid1412.080941

http://bse-atypical.blogspot.com/2008/11/transmission-of-atypical-bovine-prions.html

Wednesday, August 20, 2008

Bovine Spongiform Encephalopathy Mad Cow Disease typical and atypical strains, was there a cover-up ?

http://bse-atypical.blogspot.com/2008/08/bovine-spongiform-encephalopathy-mad.html

Tuesday, June 3, 2008 SCRAPIE USA UPDATE JUNE 2008 NOR-98 REPORTED PA

http://nor-98.blogspot.com/2008/06/scrapie-usa-update-june-2008-nor-98.html

SCRAPIE USA

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

Sunday, September 07, 2008

CWD LIVE TEST, and the political aspects or fallout of live testing for BSE in cattle in the USA

http://chronic-wasting-disease.blogspot.com/2008/09/cwd-live-test-and-political-aspects-or.html

Saturday, October 18, 2008 WYOMING STAR VALLEY MOOSE TESTS POSITIVE FOR CWD

http://chronic-wasting-disease.blogspot.com/2008/10/wyoming-star-valley-moose-tests.html

http://chronic-wasting-disease.blogspot.com/

Saturday, December 01, 2007 Phenotypic Similarity of Transmissible Mink Encephalopathy in Cattle and L-type Bovine Spongiform Encephalopathy in a Mouse Model Volume 13, Number 12-December 2007 Research

snip...see full text ;

http://transmissible-mink-encephalopathy.blogspot.com/2007/12/phenotypic-similarity-of-transmissible.html

A New Prionopathy OR more of the same old BSe and sporadic CJD

http://creutzfeldt-jakob-disease.blogspot.com/2008/08/new-prionopathy-or-more-of-same-old-bse.html

Sunday, April 20, 2008 Progress Report from the National Prion Disease Pathology Surveillance Center April 3, 2008

Atypical forms of BSE have emerged which, although rare, appear to be more virulent than the classical BSE that causes vCJD.

see full text ;

http://prionunitusaupdate2008.blogspot.com/2008/04/progress-report-from-national-prion.html

ONE HUNDRED AND FIRST MEETING OF THE SPONGIFORM ENCEPHALOPATHY ADVISORY COMMITTEE Oct 23, 2008 at 9:00 AM

http://seac992007.blogspot.com/2008/10/one-hundred-and-first-meeting-of_23.html

http://flounder068.vox.com/library/post/one-hundred-and-first-meeting-of-the-spongiform-encephalopathy-advisory-committee.html

Friday, November 21, 2008

Plasma & Serum Proteins Receive Continued FDA Approval

http://madcowfeed.blogspot.com/2008/11/plasma-serum-proteins-receive-continued.html

http://madcowfeed.blogspot.com/


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

Sunday, November 23, 2008
PRION October 8th - 10th 2008 Book of Abstracts

http://bse-atypical.blogspot.com/2008/11/prion-october-8th-10th-2008-book-of.html

TSS



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