title: Rhes deletion is neuroprotective in the 3-nitropropionic acid model of Huntington's disease.
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.
authors: Solomon H. Snyder Department of Neuroscience, Johns Hopkins University School of Medicine, Baltimore, Maryland 21205, USA.
authors: Robert G
authors: RG
authors: Mealer
authors: Srinivasa
authors: S
authors: Subramaniam
authors: Solomon H
authors: SH
authors: Snyder
mesh_terms: D000818:Animals; D004195:Disease Models, Animal; D005106:Exploratory Behavior / Q000187:drug effects / Q000502:physiology; D019204:GTP-Binding Proteins / Q000172:deficiency*; D006816:Huntington Disease / Q000139:chemically induced* / Q000235:genetics / Q000378:metabolism*; D051379:Mice; D008810:Mice, Inbred C57BL; D018345:Mice, Knockout; D009498:Neurotoxins / Q000633:toxicity*; D009574:Nitro Compounds / Q000633:toxicity*; D011422:Propionates / Q000633:toxicity*; D011597:Psychomotor Performance / Q000187:drug effects; D013385:Succinate Dehydrogenase / Q000378:metabolism
chemical_list: D009498:Neurotoxins; D009574:Nitro Compounds; D011422:Propionates; D013385:Succinate Dehydrogenase; D019204:GTP-Binding Proteins; C488189:Rasd2 protein, mouse; C015392:3-nitropropionic acid