To extend the analysis of DBT-dependent proteotoxicity from TDP-43 to another disease-associated protein, we focused on the polyglutamine (polyQ) repeat, which is a misfolded protein domain associated with neurodegenerative diseases such as Huntington’s disease (HD) and spinocerebellar ataxia (SCA) (Ross and Poirier, 2004). The gene discussed is TARDBP; the disease is autosomal dominant cerebellar ataxia.