COXFA4 and Spinocerebellar ataxia type 3: An important part of SCA3 pathogenesis comprises impairments of the mitochondria, which include altered localization of ataxin-3 inside the mitochondria, enhanced interaction of ataxin-3 82Q with mitochondrial proteins (Sdhb and Ndufa4 which are also dysregulated in our analysis), and mitochondrial DNA damage documented in SCA3 models [51, 61, 74–76].