Article
Hereditary prion protein amyloidoses.
Clinics in laboratory medicine - 1 Mar 2003
Ghetti Bernardino, Tagliavini Fabrizio, Takao M, Bugiani Orso, Piccardo Pedro
Abstract excerpt
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...
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