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Peer-reviewed veterinary case report

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

Journal:
The Journal of neuroscience : the official journal of the Society for Neuroscience
Year:
2013
Authors:
Mealer, Robert G et al.
Affiliation:
Solomon H. Snyder Department of Neuroscience · United States
Species:
rodent

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.

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Original publication: https://pubmed.ncbi.nlm.nih.gov/23447628/