Glial S100B Positive Vacuoles In Purkinje Cells: Earliest Morphological Abnormality In SCA1 Transgenic Mice.
作者： Jonathan D Fratkin , Parminder J S Vig , Jeffrey Henegar , Maripar E Lopez , Jinrong Wei
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摘要: Spinocerebellar ataxia-1 (SCA1) is caused by the expansion of a polyglutamine repeat within disease protein, ataxin-1. The overexpression mutant ataxin-1 in SCA1 transgenic mice results formation cytoplasmic vacuoles Purkinje neurons (PKN) cerebellum. PKN are closely associated with neighboring Bergmann glia. To elucidate role glia pathogenesis, cerebellar tissue from 7 days to 6 wks old and wildtype were used. We observed that glial S100B protein localized PKN. These positive began appearing much before onset behavioral abnormalities, negative for other marker proteins. Electron micrographs revealed have double membrane. In vacuoles, colocalized receptors advanced glycation end-products (RAGE), co-immunoprecipated RAGE. cultures, exogenous interacted membranes was internalized. data suggest though extrinsic sequestered into at early postnatal ages. Further, may be binding RAGE on cell these
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