Polyglutamine Spinocerebellar ataxias (SCA) are a group of genetic disorders caused by an expansion of the CAG trinucleotide in their causative genes. Currently, there is limited understanding of the underlying molecular pathogenesis alterations. Activity-induced inhibitor of death genes (AID) is a group of pro-survival genes that have been found to be neuroprotective in several neurological disorders. In this project, our goal is to explore the potential of modulating AID expression to mitigate disease progression in models of SCA2 and SCA3, the most prevalent ataxias worldwide. Our findings indicate that the activation of key transcription factors necessary for AID gene expression is reduced in SCA2 and SCA3 models. Moreover, we found impaired induction of the AID1 and AID2 genes, upon motor stimulation in disease mouse models. These findings support the hypothesis that transcriptional dysregulation of these genes might underlie SCA pathogenesis. In order to further understand the circuitry alterations associated with these disorders, we investigated whether alterations in AID gene expression was cell-type-specific. Lastly, we investigated if rescue of AID gene levels was sufficient to mitigate neuropathological hallmarks in SCA2 and SCA3 mouse models. Altogether, this work indicates that transcriptional deregulation of AID gene expression could be a promising target for SCA therapy.