NR AQAS
AU Ghetti,B.; Piccardo,P.; Frangione,B.; Bugiani,O.; Giaccone,G.; Young,K.; Prelli,F.; Farlow,M.R.; Dlouhy,S.R.; Tagliavini,F.
TI Prion protein hereditary amyloidosis - parenchymal and vascular
QU Seminars in Virology 1996; 7(N3): 189-200
PT Article
AB Prion protein (PrP) amyloidosis is a feature of Gerstmann- Sträußler-Scheinker disease (GSS) and prion protein cerebral amyloid angiopathy (PrP-CAA). GSS and PrP-CAA are associated with point mutations of the prion protein gene (PRNP); there is a broad spectrum of clinical presentations and the main signs are ataxia, spastic paraparesis, extrapyramidal signs and dementia. In GSS, parenchymal amyloid may be associated with spongiform changes or neurofibrillary lesions; in PrP- CAA, vascular amyloid is associated with neurofibrillary lesions. In the two diseases, a major component of the amyloid fibrils is a 7 kDa peptide, approximately spanning residues 81-150 of PrP.
ZR 60
SP englisch
OR Prion-Krankheiten G