ATXN3 and Spinocerebellar ataxia type 3: Indeed, KPNA3-dependent nuclear localization of ataxin-3 acts as a key event in the pathogenesis of spinocerebellar ataxia type 3 (Sowa et al., 2018), while ablation of KPNA3 in mice results in abnormal sperm morphology and influences male fertility (Miyamoto et al., 2020).