An earlier study demonstrated that, in SCA3 patients and SCA3 animal models, the level of soluble ataxin-2 is reduced and that re-establishment of ataxin-2 leads to a reduced level of mutant ataxin-3 as well as reduced behavioral defects and neuropathology in SCA3 mice [43]. Here, ATXN2 is linked to Spinocerebellar ataxia type 3.