Abstract
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.
Publication types
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Research Support, U.S. Gov't, P.H.S.
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Review
MeSH terms
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Amyloid / genetics
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Animals
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Cerebral Amyloid Angiopathy, Familial / genetics
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Cerebral Amyloid Angiopathy, Familial / pathology*
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Cerebral Amyloid Angiopathy, Familial / physiopathology
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Gerstmann-Straussler-Scheinker Disease / genetics
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Gerstmann-Straussler-Scheinker Disease / pathology*
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Gerstmann-Straussler-Scheinker Disease / physiopathology
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Humans
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Mutation
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Prion Proteins
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Prions / classification
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Prions / genetics
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Protein Precursors / genetics
Substances
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Amyloid
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PRNP protein, human
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Prion Proteins
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Prions
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Protein Precursors