Genetic analyses of ALS patients have identified more than 100 ALS-related gene variants, including many genes encoding RBPs, such as TDP-43, FUS, heterogeneous nuclear ribonucleoproteins (hnRNP) A1, hnRNPA2/B1, matrin 3 (MATR3), ataxin 2 (ATXN2), TATA-box binding protein–associated factor 15 (TAF15), T-cell–restricted intracellular antigen 1 (TIA-1), and Ewing sarcoma breakpoint region 1 (EWSR1; Al-Chalabi et al., 2017; Nguyen et al., 2018). The gene discussed is ATXN2; the disease is amyotrophic lateral sclerosis.