ATXN1 and ATXN2 were the first RBPs with a putative PrLD to be linked to the pathogenesis of neurodegenerative diseases, causing, respectively, the type 1 and type 2 spinocerebellar ataxia (Banfi et al., 1994; Lorenzetti et al., 1997), a neurodegenerative disorder characterized by an expansion of a trinucleotide CAG repeat within the coding region of the SCA1 and SCA2 genes (Banfi et al., 1994; Lorenzetti et al., 1997). Here, ATXN1 is linked to neurodegenerative disease.