ATXN2 and amyotrophic lateral sclerosis: This view is further supported by evidence that alleles of the RBP ataxin-2 (ATXN2) with intermediate repeat expansion increase the risk of ALS-FTD, as well as evidence that variants in RBPs not discussed in this review (e.g., ANG, EWS, TAF15) may contribute to the burden of ALS-FTD.