STUB1 and autosomal recessive spinocerebellar ataxia 16: Given the recessive nature of this disease in patients [12], and the lack of phenotype observed in our three rodent models of CHIP haploinsufficiency (Figs 6, 7, 8 and 9), these data suggest that either the loss or change in specific activities of CHIP drive the pathophysiologies as well as the clinical heterogeneity associated with SCAR16.