Spinocerebellar ataxia type 1 (SCA1) is a dominantly inherited and fatal neurodegenerative disease resulting from an overexpansion of CAG repeats within the Ataxin-1 (ATXN1) gene (Banfi et al., 1996; Zoghbi and Orr, 2009). Here, ATXN1 is linked to spinocerebellar ataxia type 1.