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Creutzfeldt-Jakob disease and inclusion body myositis: abundant disease-associated prion protein in muscle.


Kovacs, G G; Lindeck-Pozza, E; Chimelli, L; Araújo, A Q C; Gabbai, A A; Ströbel, T; Glatzel, M; Aguzzi, A; Budka, H (2004). Creutzfeldt-Jakob disease and inclusion body myositis: abundant disease-associated prion protein in muscle. Annals of Neurology, 55(1):121-125.

Abstract

Pathologicalprion protein (PrP(Sc)) is the hallmark of prion diseases affecting primarily the central nervous system. Using immunohistochemistry, paraffin-embedded tissue blot, and Western blot, we demonstrated abundant PrP(Sc) in the muscle of a patient with sporadic Creutzfeldt-Jakob disease and inclusion body myositis. Extraneural PrP(C)-PrP(Sc) conversion in Creutzfeldt-Jakob disease appears to become prominent when PrP(C) is abundantly available as substrate, as in inclusion body myositis muscle.

Pathologicalprion protein (PrP(Sc)) is the hallmark of prion diseases affecting primarily the central nervous system. Using immunohistochemistry, paraffin-embedded tissue blot, and Western blot, we demonstrated abundant PrP(Sc) in the muscle of a patient with sporadic Creutzfeldt-Jakob disease and inclusion body myositis. Extraneural PrP(C)-PrP(Sc) conversion in Creutzfeldt-Jakob disease appears to become prominent when PrP(C) is abundantly available as substrate, as in inclusion body myositis muscle.

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42 citations in Web of Science®
44 citations in Scopus®
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Additional indexing

Item Type:Journal Article, refereed
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:1 January 2004
Deposited On:11 Feb 2008 12:26
Last Modified:05 Apr 2016 12:21
Publisher:Wiley-Blackwell
ISSN:0364-5134
Publisher DOI:10.1002/ana.10813
PubMed ID:14705121

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