Hereditary prion protein amyloidoses

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

Research output: Contribution to journalReview article

49 Scopus citations

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.

Original languageEnglish (US)
Pages (from-to)65-85
Number of pages21
JournalClinics in Laboratory Medicine
Volume23
Issue number1
DOIs
StatePublished - Mar 1 2003

ASJC Scopus subject areas

  • Clinical Biochemistry
  • Biochemistry, medical

Fingerprint Dive into the research topics of 'Hereditary prion protein amyloidoses'. Together they form a unique fingerprint.

  • Cite this

    Ghetti, B., Tagliavini, F., Takao, M., Bugiani, O., & Piccardo, P. (2003). Hereditary prion protein amyloidoses. Clinics in Laboratory Medicine, 23(1), 65-85. https://doi.org/10.1016/S0272-2712(02)00064-1