Hereditary prion protein amyloidoses

Bernardino Ghetti, Pedro Piccardo, Orso Bugiani, Gianluigi Forloni, Michela Morbin, Mario Salmona, Fabrizio Tagliavini

Research output: Chapter in Book/Report/Conference proceedingChapter

2 Scopus citations


The term prion protein (PrP) amyloidoses is used to describe a group of diseases in which large amounts of PrP degradation products accumulate as fibrillary deposits leading to amyloid formation1,2. Accumulation of abnormal PrP isoforms occurs without significant amyloid deposition in the brain parenchyma during the course of most subacute spongiform encephalopathies, which include sporadic, familial, and iatrogenic Creutzfeldt-Jakob disease (CJD) as well as sporadic and familial fatal insomnia (FFI)3-6. Typically, PrP-amyloid is seen in Gerstmann-Sträussler-Scheinker disease (GSS), PrP cerebral amyloid angiopathy (PrP-CAA), variant CJD (vCJD), and kuru 1-3.

Original languageEnglish (US)
Title of host publicationNeurodegeneration and Prion Disease
PublisherSpringer US
Number of pages27
ISBN (Print)0387239227, 9780387239224
StatePublished - Dec 1 2005


ASJC Scopus subject areas

  • Neuroscience(all)

Cite this

Ghetti, B., Piccardo, P., Bugiani, O., Forloni, G., Morbin, M., Salmona, M., & Tagliavini, F. (2005). Hereditary prion protein amyloidoses. In Neurodegeneration and Prion Disease (pp. 83-109). Springer US.