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

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.

• Authors: Gabor G Kovacs, Elisabeth Lindeck-Pozza, Leila Chimelli, Abelardo Q C Araújo, Alberto A Gabbai, Thomas Ströbel, Markus Glatzel, Adriano Aguzzi, Herbert Budka
• Journal: Annals of neurology (Ann Neurol) Vol. 55 Issue 1 Pg. 121-5 (Jan 2004) ISSN: 0364-5134 [Print] United States
• PMID: 14705121 (Publication Type: Case Reports, Comparative Study, Journal Article, Research Support, Non-U.S. Gov't)

Chemical References

• PrPC Proteins
• PrPSc Proteins

Topics

• Aged
• Blotting, Western
• Brain (metabolism, pathology)
• Creutzfeldt-Jakob Syndrome (complications, metabolism, pathology)
• Humans
• Immunohistochemistry
• Male
• Muscle, Skeletal (metabolism, pathology)
• Myositis, Inclusion Body (complications, metabolism, pathology)
• Polymerase Chain Reaction
• PrPC Proteins (metabolism)
• PrPSc Proteins (metabolism)