There are 10 RBPs with known ALS mutations in their encoding genes: ANG, EWSR1, Fused in sarcoma (FUS), hnRNPA1, hnRNPA2B1, RGNEF, SETX, TAF15, TIA-1 and TAR DNA binding protein 43 (TDP-43; Table 1). This evidence concerns the gene FUS and amyotrophic lateral sclerosis.