Several therapeutic strategies are under study for MJD treatment such as modulation of Ca2+ signalling, inhibition of calpain-mediated proteolysis of mutant ataxin-3 in the brain or promotion of degradation of mutant ataxin-3 species, either by activation of the proteasome or of the beclin-1 autophagy pathway [14]–[17]. The gene discussed is ATXN3; the disease is Machado-Joseph disease.