Importantly, our study also shows increased expression of Ephrin-A3 at both the RNA and protein level in the brainstem of Atxn3-KO mice, supporting the view that the absence of ATXN3 leads to dysregulation of this signaling pathway in a disease-relevant brain region and suggesting that Ephrin-A3 is a compelling candidate for further study in SCA3 disease model systems. This evidence concerns the gene ATXN3 and Spinocerebellar ataxia type 3.