Hereditary prion protein amyloidoses

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

Research output: Contribution to journalArticle

49 Citations (Scopus)

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

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Amyloidosis
Neurofibrillary Tangles
Prion Diseases
Amyloid
Cerebral Amyloid Angiopathy
Endopeptidase K
Amyloidogenic Proteins
Blood Vessels
Prions
Protein Isoforms
Brain
Prion Proteins

ASJC Scopus subject areas

  • Medicine(all)
  • Biochemistry, Genetics and Molecular Biology(all)

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

Hereditary prion protein amyloidoses. / Ghetti, Bernardino; Tagliavini, Fabrizio; Takao, M.; Bugiani, Orso; Piccardo, Pedro.

In: Clinics in Laboratory Medicine, Vol. 23, No. 1, 01.03.2003, p. 65-85.

Research output: Contribution to journalArticle

Ghetti, B, Tagliavini, F, Takao, M, Bugiani, O & Piccardo, P 2003, 'Hereditary prion protein amyloidoses', Clinics in Laboratory Medicine, vol. 23, no. 1, pp. 65-85. https://doi.org/10.1016/S0272-2712(02)00064-1
Ghetti, Bernardino ; Tagliavini, Fabrizio ; Takao, M. ; Bugiani, Orso ; Piccardo, Pedro. / Hereditary prion protein amyloidoses. In: Clinics in Laboratory Medicine. 2003 ; Vol. 23, No. 1. pp. 65-85.
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