Prion protein hereditary amyloidosis: Parenchymal and vascular

Bernardino Ghetti, Pedro Piccardo, Blas Frangione, Orso Bugiani, Giorgio Giaccone, Katherine Young, Frances Prelli, Martin R. Farlow, Stephen R. Dlouhy, Fabrizio Tagliavini

Research output: Contribution to journalArticlepeer-review


Prion protein (PrP) amyloidosis is a feature of Gerstmann-Straussler-Scheinker disease (GSS) and prion protein cerebral amyloid angiopathy (PrP-CAA). GSS and PrP-CAA are associated with point mutations of the prion protein gene (PRNP); there is a broad spectrum of clinical presentations and the main signs are ataxia, spastic paraparesis, extrapyramidal signs and dementia. In GSS, parenchymal amyloid may be associated with spongiform changes or neurofibrillary lesions; in PrP-CAA, vascular amyloid is associated with neurofibrillary lesions. In the two diseases, a major component of the amyloid fibrils is a 7 kDa peptide, approximately spanning residues 81-150 of Prp.

Original languageEnglish
Pages (from-to)189-200
Number of pages12
JournalSeminars in Virology
Issue number3
Publication statusPublished - Jun 1996


  • Amyloid
  • Cerebrovascular amyloidosis
  • Gerstmann-Straussler-Scheinker disease
  • Prion protein
  • PRNP gene

ASJC Scopus subject areas

  • Immunology
  • Virology


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