This proteolysis is associated with neurotoxicity and inhibition of caspases has been shown to be beneficial in different disease models of SCA3 (Berke et al., 2004), DRPLA (Ellerby et al., 1999), SCA7 (Garden et al., 2002) or HD (Graham et al., 2006). Here, ATXN7 is linked to Huntington disease.