Hereditary Prion Protein Amyloidoses

Overview

Journal Clin Lab Med

Specialty Pathology

Date 2003 May 8

PMID 12733425

Citations 31

Authors

Bernardino Ghetti

Fabrizio Tagliavini

M Takao

Orso Bugiani

Pedro Piccardo

Affiliations

Soon will be listed here.

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.

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