We had the opportunity to work with a transgenic mouse model of SCA-3 generated by Silva-Fernandes et al. (2014), which reproduces many of the neurological and neuropathological signs of the disease, following a gradual and progressive pattern with expression of mutant ataxin-3 at near endogenous levels. The gene discussed is ATXN3; the disease is Spinocerebellar ataxia type 3.