Interestingly, ATXN2 with intermediate PolyQ repeats is associated not only with spinocerebellar ataxia type 2 (SCA2), but also with ALS (Elden et al., 2010), serving as a risk factor for C9ORF72-linked ALS (Zhang et al., 2017). Here, C9orf72 is linked to amyotrophic lateral sclerosis.