This chaperone has been found previously in a genetic screen to reduce the neurotoxicity of the poly-glutamine containing protein ataxin 3 associated with spinocerebellar ataxia type 3 (SCA3), a disease that exhibits a similar cytoplasmic aggregation phenotype to the TDP-43 proteinopathies [26], [27]. Here, ATXN3 is linked to Spinocerebellar ataxia type 3.