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2020-05-03
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CREUTZFELDT-JAKOB DISEASE AND INCLUSION BODY MYOSITIS: ABUNDANT DISEASE-ASSOCIATED PRION PROTEIN IN MUSCLE.
Kovacs, G. G. et al. | Date Issued: 2004
Author
Kovacs, G. G.
Pozza, E. L.
Chimelli, L. C.
Araújo, A. Q. C.
Gabbai, A. A.
Strobel, T.
Glatzel, M.
Aguzzi, A.
Budka, H.
Affilliation
University of Vienna. Institute of Neurology. Vienna, Austria / Austrian Reference Centre for Human Prion Diseases. Vienna, Austria / National Institute of Psychiatry and Neurology. Budapest, Hungary.
University of Vienna. Institute of Neurology. Vienna, Austria / Austrian Reference Centre for Human Prion Diseases. Vienna, Austria.
Federal University of Rio de Janeiro. School of Medicine. Department of Pathology. Rio de Janeiro, RJ, Brazil.
Federal University of Rio de Janeiro. School of Medicine. Department of Neurology. Rio de Janeiro, RJ, Brazil.
Federal University of São Paulo. School of Medicine. Department of Neurology. São Paulo, SP, Brazil.
University of Vienna. Institute of Neurology. Vienna, Austria / Austrian Reference Centre for Human Prion Diseases. Vienna, Austria.
University Hospital of Zürich. Institute of Neuropathology. Zürich, Switzerland.
University Hospital of Zürich. Institute of Neuropathology. Zürich, Switzerland.
University of Vienna. Institute of Neurology. Vienna, Austria / Austrian Reference Centre for Human Prion Diseases. Vienna, Austria.
Abstract
Pathological prion protein (PrPSc) is the hallmark ofprion diseases affecting primarily the central nervous sys-tem. Using immunohistochemistry, paraffin-embeddedtissue blot, and Western blot, we demonstrated abundant PrPScin the muscle of a patient with sporadicCreutzfeldt–Jakob disease and inclusion body myositis. Extraneural PrPC-PrPScconversion in Creutzfeldt–Jakobdisease appears to become prominent when PrPCis abun-dantly available as substrate, as in inclusion body myosi-tis muscle.
Keywords
Creutzfeldt-Jakob disease
Myositis
Prion protein in muscle
 
Publisher
Wiley
Citation
KOVACS, Gabor G. et al. Creutzfeldt-Jakob disease and inclusion body myositis: abundant disease-associated prion protein in muscle. Annals of Neurology, v. 55, p. 121-125, 2004.
DOI
10.1002/ana.10813
ISSN
0364-5134
Notes
Abelardo de Queiroz Campos Araújo. Fundação Oswaldo Cruz. Instituto Nacional de Infectologia Evandro Chagas. Documento produzido em parceria ou por autor vinculado à Fiocruz, mas não consta a informação no documento.