Thus we used dominant models of ataxias (SCA1, SCA3, SCA7) and FTLD (TDP-43, FUS, TAU) where pathological or control proteins can be targeted to neurons in a temporally controlled manner with the RU486 inducible ElavGal4GS line (see below for complete genotype description). The gene discussed is ATXN3; the disease is Ataxia.