NR AEYI
AU Guiroy,D.C.; Wakayama,I.; Liberski,P.P.; Gajdusek,D.C.
TI Relationship of microglia and scrapie amyloid-immunoreactive plaques in kuru, Creutzfeldt-Jakob disease and Gerstmann-Sträussler syndrome
QU Acta Neuropathologica 1994 May; 87(5): 526-30
PT journal article
AB Kuru, Creutzfeldt-Jakob disease (CJD) and Gerstmann-Sträussler syndrome (GSS) are transmissible dementias affecting humans characterized neuropathologically by intraneuronal vacuolation, spongiform change, astrocytic hypertrophy and hyperplasia and the variable presence of amyloid plaques. It has been suggested that microglia are amyloid-forming cells, which play an essential role in amyloid plaque formation. To study the relationship between microglia and amyloid plaques in kuru, CJD and GSS, cerebellar tissues were examined by the double-immunostaining technique using anti-ferritin antibodies as the microglial marker and anti-scrapie amyloid antibody as plaque marker. Ferritin-immunoreactive microglia were observed interdigitating with and among unicentric, multicentric and diffuse types of scrapie amyloid-immunoreactive plaques and were found to a lesser extent in the neuropil. In kuru and CJD, scrapie amyloid-immunoreactive plaques were predominantly unicentric and were observed in the granular layer. In kuru, 53% of the amyloid plaques were associated with microglia, whereas only 30% of plaques in CJD were. In contrast, scrapie-amyloid-immunoreactive plaques in GSS were of the multicentric type, predominantly observed in the molecular layer, and 90% of these plaques were associated with microglia. Our data indicate that microglia are frequently associated with scrapie amyloid-immunoreactive plaques in GSS, less commonly in kuru and to a much lesser extent in CJD, suggesting that microglia may play a variable but important role in the formation of plaques in the transmissible spongiform encephalopathies.
IN Die beim Menschen bekannten übertragbaren spongiformen Enzephalopathien unterscheiden sich in der Verteilung der amyloiden Plaques im Gehirn. Bei der Creutzfeldt-Jakob-Krankheit sind 30% der Prionproteinaggregate mit Mikrogliazellen assoziiert. Bei Kuru sind es 53% und beim Gerstmann-Sträussler-Syndrom 90%.
MH Adolescent; Aged; Amyloid/*metabolism; Cerebellum/metabolism/pathology; Creutzfeldt-Jakob Syndrome/*metabolism/pathology; Female; Ferritin/metabolism; Gerstmann-Sträussler-Scheinker Disease/*metabolism/pathology; Human; Immunohistochemistry; Kuru/*metabolism/pathology; Male; Microglia/*metabolism/ultrastructure; Middle Age; Prions/*metabolism
AD Laboratory of Central Nervous System Studies, NINDS, Frederick Cancer Research and Development Center, MD 21702.
SP englisch
PO Deutschland