Immunoblotting of protein lysates of the cerebellar tissue from MJD mice dissected at autopsy at 18 weeks revealed human ataxin-3 protein expression in the MJD mice as well as endogenous ataxin-3 in both the MJD mice and non-transgenic controls (Fig. 4A). The gene discussed is ATXN3; the disease is Spinocerebellar ataxia type 3.