This has been shown to be a viable approach in several animal models of SCA1 and SCA3 (Machado-Joseph disease), in which protein aggregates, cellular degeneration, thinning of the molecular layer of the cerebellum and motor deficits can be prevented upon silencing of the gene underlying the different forms of SCAs (ATXN1 and ATXN3 for SCA1 and 3, respectively) [6,102,103,104]. Here, ATXN3 is linked to Machado-Joseph disease.