NR AOCR
AU Ghetti,B.; Tagliavini,F.; Takao,M.; Bugiani,O.; Piccardo,P.
TI Hereditary prion protein amyloidoses
QU Clinics in Laboratory Medicine 2003 Mar; 23(1): 65-85, viii
PT journal article; review; review, tutorial
AB Prion protein (PrP) amyloid accumulation is the pathologic hallmark of some inherited prion diseases such as Gerstmann-Sträussler-Scheinker disease (GSS) and PrP cerebral amyloid angiopathy (PrP-CAA). In GSS, parenchymal amyloidosis may coexist with spongiform degeneration or neurofibrillary tangles, whereas in PrP-CAA, vascular amyloid coexists with neurofibrillary tangles. In GSS, N-truncated and C-truncated proteinase K-resistant PrP isoforms are present in the brain.
ZR 63
MH Amyloid/genetics; Animals; Cerebral Amyloid Angiopathy, Familial/genetics/*pathology/physiopathology; Gerstmann-Sträussler-Scheinker Disease/genetics/*pathology/physiopathology; Human; Mutation; Prions/classification/genetics; Protein Precursors/genetics; Support, U.S. Gov't, P.H.S.
AD Department of Pathology and Laboratory Medicine and Indiana Alzheimer Disease Center, Indiana University School of Medicine, 635 Barnhill Drive, MS A128, Indianapolis, IN 46202-5120, USA. bghetti@iupui.edu
SP englisch
PO USA