title: Beta PP participates in PrP-amyloid plaques of Gerstmann-Sträussler-Scheinker disease, Indiana kindred.
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 beta PP and A beta immunoreactivities associated with PrP-amyloid deposits and degenerating neurites. In one asymptomatic individual with PrP-amyloid deposits, Alz50 and A beta 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 beta PP. In one symptomatic, senile patient, A beta 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 beta PP, synaptophysin and ubiquitinated material most probably revealed a reaction of neurites to PrP-amyloid, whereas the extracellular deposition of A beta was likely an age-related phenomenon.
authors.affiliation: Istituto Neurologico Carlo Besta, Milan, Italy.
authors.forename: O
authors.initials: O
authors.lastname: Bugiani
authors.forename: G
authors.initials: G
authors.lastname: Giaccone
authors.forename: L
authors.initials: L
authors.lastname: Verga
authors.forename: B
authors.initials: B
authors.lastname: Pollo
authors.forename: B
authors.initials: B
authors.lastname: Frangione
authors.forename: M R
authors.initials: MR
authors.lastname: Farlow
authors.forename: F
authors.initials: F
authors.lastname: Tagliavini
authors.forename: B
authors.initials: B
authors.lastname: Ghetti
mesh_terms: D000368:Aged; D016229:Amyloid beta-Peptides / Q000032:analysis; D016564:Amyloid beta-Protein Precursor / Q000032:analysis*; D002540:Cerebral Cortex / Q000737:chemistry / Q000473:pathology; D003704:Dementia / Q000235:genetics / Q000378:metabolism / Q000473:pathology; D005260:Female; D016098:Gerstmann-Straussler-Scheinker Disease / Q000235:genetics / Q000378:metabolism* / Q000473:pathology; D006801:Humans; D008297:Male; D008875:Middle Aged; D016501:Neurites / Q000737:chemistry; D018620:PrPSc Proteins; D011328:Prions / Q000032:analysis*
chemical_list: D016229:Amyloid beta-Peptides; D016564:Amyloid beta-Protein Precursor; D018620:PrPSc Proteins; D011328:Prions