Prion protein hereditary amyloidosis

Parenchymal and vascular

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

Research output: Contribution to journalArticle

9 Citations (Scopus)

Abstract

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
Volume7
Issue number3
DOIs
StatePublished - Jun 1996

Fingerprint

Familial Amyloidosis
Gerstmann-Straussler-Scheinker Disease
Blood Vessels
Amyloid
Spastic Paraparesis
Cerebral Amyloid Angiopathy
Amyloidosis
Ataxia
Point Mutation
Dementia
Prion Proteins
Peptides
Genes

Keywords

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

ASJC Scopus subject areas

  • Immunology
  • Virology

Cite this

Ghetti, B., Piccardo, P., Frangione, B., Bugiani, O., Giaccone, G., Young, K., ... Tagliavini, F. (1996). Prion protein hereditary amyloidosis: Parenchymal and vascular. Seminars in Virology, 7(3), 189-200. https://doi.org/10.1006/smvy.1996.0024

Prion protein hereditary amyloidosis : Parenchymal and vascular. / Ghetti, Bernardino; Piccardo, Pedro; Frangione, Blas; Bugiani, Orso; Giaccone, Giorgio; Young, Katherine; Prelli, Frances; Farlow, Martin; Dlouhy, Stephen; Tagliavini, Fabrizio.

In: Seminars in Virology, Vol. 7, No. 3, 06.1996, p. 189-200.

Research output: Contribution to journalArticle

Ghetti, B, Piccardo, P, Frangione, B, Bugiani, O, Giaccone, G, Young, K, Prelli, F, Farlow, M, Dlouhy, S & Tagliavini, F 1996, 'Prion protein hereditary amyloidosis: Parenchymal and vascular', Seminars in Virology, vol. 7, no. 3, pp. 189-200. https://doi.org/10.1006/smvy.1996.0024
Ghetti B, Piccardo P, Frangione B, Bugiani O, Giaccone G, Young K et al. Prion protein hereditary amyloidosis: Parenchymal and vascular. Seminars in Virology. 1996 Jun;7(3):189-200. https://doi.org/10.1006/smvy.1996.0024
Ghetti, Bernardino ; Piccardo, Pedro ; Frangione, Blas ; Bugiani, Orso ; Giaccone, Giorgio ; Young, Katherine ; Prelli, Frances ; Farlow, Martin ; Dlouhy, Stephen ; Tagliavini, Fabrizio. / Prion protein hereditary amyloidosis : Parenchymal and vascular. In: Seminars in Virology. 1996 ; Vol. 7, No. 3. pp. 189-200.
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AU - Farlow, Martin

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