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

First Author  Mealer RG Year  2013
Journal  J Neurosci Volume  33
Issue  9 Pages  4206-10
PubMed ID  23447628 Mgi Jnum  J:196058
Mgi Id  MGI:5486506 Doi  10.1523/JNEUROSCI.3730-12.2013
Citation  Mealer RG, et al. (2013) Rhes deletion is neuroprotective in the 3-nitropropionic acid model of Huntington's disease. J Neurosci 33(9):4206-10
abstractText  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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