Creutzfeldt-Jakob disease and inclusion body myositis: abundant disease-associated prion protein in 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.
Kovacs
Gabor G
GG
Institute of Neurology, University of Vienna, and Austrian Reference Centre for Human Prion Diseases, Vienna, Austria.
Lindeck-Pozza
Elisabeth
E
Chimelli
Leila
L
Araújo
Abelardo Q C
AQ
Gabbai
Alberto A
AA
Ströbel
Thomas
T
Glatzel
Markus
M
Aguzzi
Adriano
A
Budka
Herbert
H
D000368:Aged; D015153:Blotting, Western; D001921:Brain / Q000378:metabolism / Q000473:pathology*; D007562:Creutzfeldt-Jakob Syndrome / Q000150:complications / Q000378:metabolism / Q000473:pathology*; D006801:Humans; D007150:Immunohistochemistry; D008297:Male; D018482:Muscle, Skeletal / Q000378:metabolism / Q000473:pathology*; D018979:Myositis, Inclusion Body / Q000150:complications / Q000378:metabolism / Q000473:pathology*; D016133:Polymerase Chain Reaction; D018621:PrPC Proteins / Q000378:metabolism; D018620:PrPSc Proteins / Q000378:metabolism*
D018621:PrPC Proteins; D018620:PrPSc Proteins