Hereditary prion protein amyloidoses

Bernardino Ghetti, Fabrizio Tagliavini, M. Takao, Orso Bugiani, Pedro Piccardo

Research output: Contribution to journalReview articlepeer-review

49 Scopus citations


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.

Original languageEnglish (US)
Pages (from-to)65-85
Number of pages21
JournalClinics in Laboratory Medicine
Issue number1
StatePublished - Mar 1 2003

ASJC Scopus subject areas

  • Clinical Biochemistry
  • Biochemistry, medical

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