NR AIIC
AU Michel,B.
TI [Encephalopathy caused by prions]
OT Les encephalopathies dues aux prions
QU Revue Neurologique 1990; 146(1): 1-11
PT journal article; review; review, tutorial
AB The concept of prion encephalopathy has emerged from such previous notions as slow virus infections, spongiform encephalopathies or transmissible dementias. The term prion (Prusiner, 1982) is now used in preference to unconventional agents. Proteins and genes of prions have recently been identified by molecular biology. Exactly how prion proteins are amplified in cells is still unknown. It has been demonstrated that amyloid deposits in scrapie-infected brain, Creutzfeldt-Jakob, Gerstmann-Sträussler and Kuru diseases are composed of prion proteins. Prion encephalopathies are good models to study some immunopathological mechanisms observed in the central nervous system in degenerative diseases or ageing.
ZR 94
MH Amyloidosis/complications; Animal; Creutzfeldt-Jakob Syndrome/*etiology/pathology/physiopathology; English Abstract; Human; Kuru/*etiology/pathology/physiopathology; *Prions; Ruminants; Scrapie/*etiology/pathology/physiopathology
AD Service de Neurologie, Hopital Sainte-Marguerite, Marseille.
SP französisch
PO Frankreich