βPP participates in PrP-amyloid plaques of gerstmann-sträussler-scheinker disease, Indiana kindred

Orso Bugiani, Giorgio Giaccone, Laura Verga, Bianca Pollo, Blas Frangione, Martin Farlow, Fabrizio Tagliavini, Bernardino Ghetti

Research output: Contribution to journalArticle

52 Citations (Scopus)

Abstract

Gerstmann-Sträussler-Scheinker disease in the Indiana kindred is pathologically characterized by deposits of PrP-amyloid, neurofibrillary tangles and degenerating neurites. The aim of this study was to investigate seven patients of different ages for βPP and Aβ immunoreactivities associated with PrP-amyloid deposits and degenerating neurites. In one asymptomatic individual with PrP-amyloid deposits, Alz50 and Aβ immunoreactivities were absent. In six symptomatic patients, the degenerating neurites surrounding PrP-amyloid deposits were labeled by Alz50 and by antibodies to synaptophysin, ubiquitin and the N- and C-terminal domains of βPP. In one symptomatic, senile patient, Aβ immunoreactivity was present in the extracellular space, often in association with PrP-amyloid deposits. The analysis of the immunohistochemical findings suggested that in the Indiana kindred the intracellular accumulation of βPP, synaptophysin and ubiquitinated material most probably revealed a reaction of neurites to PrP-amyloid, whereas the extracellular deposition of Aβ was likely an age-related phenomenon.

Original languageEnglish
Pages (from-to)64-70
Number of pages7
JournalJournal of Neuropathology and Experimental Neurology
Volume52
Issue number1
StatePublished - 1993

Fingerprint

Amyloid Plaques
Neurites
Synaptophysin
Ubiquitin C
Neurofibrillary Tangles
Extracellular Space
Amyloid
Antibodies

Keywords

  • Alzheimer’s disease
  • Amyloid
  • Gerstmann-Sträussler-Scheinker disease
  • PrP-amyloid
  • βPP

ASJC Scopus subject areas

  • Clinical Neurology
  • Pathology and Forensic Medicine
  • Cellular and Molecular Neuroscience
  • Neurology
  • Neuroscience(all)

Cite this

βPP participates in PrP-amyloid plaques of gerstmann-sträussler-scheinker disease, Indiana kindred. / Bugiani, Orso; Giaccone, Giorgio; Verga, Laura; Pollo, Bianca; Frangione, Blas; Farlow, Martin; Tagliavini, Fabrizio; Ghetti, Bernardino.

In: Journal of Neuropathology and Experimental Neurology, Vol. 52, No. 1, 1993, p. 64-70.

Research output: Contribution to journalArticle

Bugiani, Orso ; Giaccone, Giorgio ; Verga, Laura ; Pollo, Bianca ; Frangione, Blas ; Farlow, Martin ; Tagliavini, Fabrizio ; Ghetti, Bernardino. / βPP participates in PrP-amyloid plaques of gerstmann-sträussler-scheinker disease, Indiana kindred. In: Journal of Neuropathology and Experimental Neurology. 1993 ; Vol. 52, No. 1. pp. 64-70.
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