The potassium channel subfamily Kv3 consists of four subunits which are encoded by KCNC1, KCNC2, KCNC3, and KCNC4. Pathogenic variants in KCNC3 are associated with spinocerebellar ataxia type 13, whereas KCNC2 and KCNC4 have not been associated with human disease up to this moment [8]. Here, KCNA3 is linked to spinocerebellar ataxia type 13.