In total, our data support our previous findings that highlight the role of aberrant ubiquitination in the pathogenesis of SCAR16; however, the loss of CHIP ubiquitin ligase activity alone may not fully explain the molecular mechanisms underlying the diverse pathophysiology observed in the heterogeneity of SCAR16 disease. The gene discussed is STUB1; the disease is autosomal recessive spinocerebellar ataxia 16.