Rhes deletion is neuroprotective in the 3-nitropropionic acid model of Huntington's disease

Robert G. Mealer, Srinivasa Subramaniam, Solomon H. Snyder

Research output: Contribution to journalArticlepeer-review

Abstract

Although the mutated protein causing Huntington's disease (HD) is expressed throughout the body, the major pathology of HD is localized to the striatum of the brain. We previously reported that the striatal-enriched protein Rhes binds the mutated huntingtin protein and enhances its cytotoxicity. We now demonstrate that Rhes-deleted mice are dramatically protected from neurotoxicity and motor dysfunction in a striatal-specific model of HD elicited by 3-nitropropionic acid. This finding suggests that Rhes may, in part, determine the striatal selectivity of HD.

Original languageEnglish (US)
Pages (from-to)4206-4210
Number of pages5
JournalJournal of Neuroscience
Volume33
Issue number9
DOIs
StatePublished - Feb 27 2013

ASJC Scopus subject areas

  • Neuroscience(all)

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