Ectopic expression of the striatal-enriched GTPase Rhes elicits cerebellar degeneration and an ataxia phenotype in Huntington's disease

Summary of the graphical abstract for the Role of Rhes in Huntington's disease (HD). (A) Rhes deleted N171-82Q transgenic (Rhes KO/N171-82Q) mice are protected from HD-related behavioral and anatomical deficits, whereas Rhes overexpression in the cerebellum of N171-82Q mice potentiates HD-related deficit and induces ataxia-like phenotype. (B) Hdh150Q/150Q knock-in HD mice develops late onset, but reintroducing Rhes into Rhes deleted Hdh150Q/150Q (Rhes KO/Hdh150Q/150Q) mice striatum elicits rapid HD onset, such as rotarod defects.325