ATAXIN-2 (ATXN2) Regulates RGS8 Expression Via RNA Interaction (P1.045)

CONCLUSIONS: Mutant ATXN2 reduced RGS8 mRNA steady-state levels, and RGS protein levels to an even greater extent. As we showed interaction of ATXN2 and RGS8 mRNA, our data support a role for ATXN2 in mRNA translation. As knockout of the related RGS6 causes severe ataxia in mice, our results implicate RGS family members in the pathogenesis of polyQ-mediated neurodegeneration in SCA2 and point to new therapeutic targets. Study Supported by: Grants RO1NS33123 and RC4NS073009 from the National Institutes of Neurological Disorders and Stroke to SMP.Disclosure: Dr. Paul has nothing to disclose. Dr. Dansithong has nothing to disclose. Dr. Pulst has received personal compensation for activities with Athena Diagnostics. Dr. Pulst has received license or royalty payments from Cedars-Sinai Medical Center.
Source: Neurology - Category: Neurology Authors: Tags: Movement Disorders: Basic Mechanisms Source Type: research