Journal
SCIENCE
Volume 324, Issue 5932, Pages 1327-1330Publisher
AMER ASSOC ADVANCEMENT SCIENCE
DOI: 10.1126/science.1172871
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Funding
- USPHS [MH18501]
- Research Scientist Award [DA00074]
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Huntington's disease (HD) is caused by a polyglutamine repeat in the protein huntingtin (Htt) with mutant Htt (mHtt) expressed throughout the body and similarly in all brain regions. Yet, HD neuropathology is largely restricted to the corpus striatum. We report that the small guanine nucleotide-binding protein Rhes, which is localized very selectively to the striatum, binds physiologically to mHtt. Using cultured cells, we found Rhes induces sumoylation of mHtt, which leads to cytotoxicity. Thus, Rhes-mHtt interactions can account for the localized neuropathology of HD.
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