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- Title
Rhes Deletion Is Neuroprotective in the 3-Nitropropionic Acid Model of Huntington's Disease.
- Authors
Mealer, Robert G.; Subramaniam, Srinivasa; Snyder, Solomon H.
- 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.
- Subjects
HUNTINGTON disease; PROPIONIC acid; NEUROPROTECTIVE agents; GENETIC mutation; GENE expression; CELL-mediated cytotoxicity
- Publication
Journal of Neuroscience, 2013, Vol 33, Issue 9, p4206
- ISSN
0270-6474
- Publication type
Article
- DOI
10.1523/JNEUROSCI.3730-12.2013