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Rhes, a striatal specific protein, mediates mutant-huntingtin cytotoxicity.

Authors: Subramaniam, S  Sixt, KM  Barrow, R  Snyder, SH 
Citation: Subramaniam S, etal., Science. 2009 Jun 5;324(5932):1327-30. doi: 10.1126/science.1172871.
Pubmed: (View Article at PubMed) PMID:19498170
DOI: Full-text: DOI:10.1126/science.1172871

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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CRRD Object Information
CRRD ID: 8554673
Created: 2014-05-08
Species: All species
Last Modified: 2014-05-08
Status: ACTIVE


RGD is funded by grant HL64541 from the National Heart, Lung, and Blood Institute on behalf of the NIH.