Atx2, an evolutionarily conserved RBP [17,18], has been identified as a genetic determinant and risk factor for several diseases, such as spinocerebellar ataxia type II (SCA2) and amyotrophic lateral sclerosis (ALS) [9,11,19,20,21,22,23,24,25,26,27,28,29,30,31]. This evidence concerns the gene ATXN2 and amyotrophic lateral sclerosis.