NR ACAA
AU Bugiani,O.; Giaccone,G.; Verga,L.; Pollo,B.; Frangione,B.; Farlow,M.R.; Tagliavini,F.; Ghetti,B.
TI Beta PP participates in PrP-amyloid plaques of Gerstmann-Sträussler-Scheinker disease, Indiana kindred
QU Journal of Neuropathology and Experimental Neurology 1993 Jan; 52(1): 64-70
PT journal article
AB 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.
IN Die Ablagerung von Prionprotein in von der Gerstmann-Sträussler-Scheinker-Krankheit betroffenen PatienInnen mit dem Krankheitstyp Indiana wird von einer Akkumulation von Synaptophysin und Ubiquitin in Neuronen begleitet.
MH Aged; Amyloid beta-Protein/analysis; Amyloid beta-Protein Precursor/*analysis; Cerebral Cortex/chemistry/pathology; Dementia/genetics/metabolism/pathology; Female; Gerstmann-Sträussler-Scheinker Disease/genetics/*metabolism/pathology; Human; Male; Middle Age; Neurites/chemistry; PrPsc Proteins; Prions/*analysis; Support, Non-U.S. Gov't; Support, U.S. Gov't, P.H.S.
AD Istituto Neurologico Carlo Besta, Milan, Italy.
SP englisch
PO USA