A precise mapping of these cleavage sites, followed by a further analysis of the originating fragments and their disease-relevant properties, might give important insight into the role of these fragments in the molecular pathogenesis of SCA17, as previously conducted for huntingtin in HD and of ataxin-3 in MJD [26, 50]. This evidence concerns the gene ATXN3 and spinocerebellar ataxia type 17.