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The role of PrP in pathogenesis of experimental scrapie.


Weissmann, C; Fischer, M; Raeber, A; Büeler, H; Sailer, A; Shmerling, D; Rülicke, T; Brandner, S; Aguzzi, A (1996). The role of PrP in pathogenesis of experimental scrapie. Cold Spring Harbor Symposia on Quantitative Biology, 61:511-522.

Abstract

It was recognized early on that the agent responsible for transmissible spongiform encephalopathies such as scrapie in sheep or kuru, Creutzfeldt-Jakob disease (CJD), Gerstmann-Sträussler-Scheinker disease (GSS), and fatal familial insomnia (FFI) in man had quite extraordinary properties, such as unusually long incubation periods (measured in months to years) and uncommon resistance to high temperature, formaldehyde treatment, and UV irradiation. The agent has been designated as “prion” (see glossary, Table 1) to distinguish it from conventional pathogens such as bacteria and viruses (Prusiner 1982).

In recent years, a new form of prion disease emerged in Great Britain and to a lesser extent in other European countries, namely, bovine spongiform encephalopathy (BSE) or mad cow disease, which has been attributed to the consumption by cattle of feed supplements derived from scrapie-contaminated sheep and later from cattle offal (Wilesmith et al. 1992). It is, however, quite possible that BSE originated as a sporadic.

It was recognized early on that the agent responsible for transmissible spongiform encephalopathies such as scrapie in sheep or kuru, Creutzfeldt-Jakob disease (CJD), Gerstmann-Sträussler-Scheinker disease (GSS), and fatal familial insomnia (FFI) in man had quite extraordinary properties, such as unusually long incubation periods (measured in months to years) and uncommon resistance to high temperature, formaldehyde treatment, and UV irradiation. The agent has been designated as “prion” (see glossary, Table 1) to distinguish it from conventional pathogens such as bacteria and viruses (Prusiner 1982).

In recent years, a new form of prion disease emerged in Great Britain and to a lesser extent in other European countries, namely, bovine spongiform encephalopathy (BSE) or mad cow disease, which has been attributed to the consumption by cattle of feed supplements derived from scrapie-contaminated sheep and later from cattle offal (Wilesmith et al. 1992). It is, however, quite possible that BSE originated as a sporadic.

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Additional indexing

Item Type:Journal Article, refereed, original work
Communities & Collections:04 Faculty of Medicine > University Hospital Zurich > Institute of Neuropathology
Dewey Decimal Classification:570 Life sciences; biology
610 Medicine & health
Language:English
Date:1996
Deposited On:11 Feb 2008 12:25
Last Modified:05 Apr 2016 12:20
Publisher:Cold Spring Harbor Laboratory Press
ISSN:0091-7451
Publisher DOI:10.1101/SQB.1996.061.01.051
PubMed ID:9246477

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