SCA48, the most recently described SCA, is an autosomal dominant disease caused by mutation of the STIP1 homology and U box-containing 1 (STUB1) gene (1, 2, 3, 4, 5, 6, 7, 8, 9, 10, 11, 12, 13, 14, 15, 16). Here, STIP1 is linked to autosomal dominant cerebellar ataxia.