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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.

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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.


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
Date:1 January 2004
Deposited On:11 Feb 2008 12:26
Last Modified:05 Apr 2016 12:21
Publisher DOI:10.1002/ana.10813
PubMed ID:14705121

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