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

Srinivasa Subramaniam, Katherine M. Sixt, Roxanne Barrow, Solomon H. Snyder

Research output: Contribution to journalArticlepeer-review

233 Scopus citations


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.

Original languageEnglish (US)
Pages (from-to)1327-1330
Number of pages4
Issue number5932
StatePublished - 2009

ASJC Scopus subject areas

  • General


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