Here, we focus on several RBPs that are known to be mutated in patients with ALS: TAR DNA-binding protein 43 (TDP-43), heterogeneous nuclear ribonucleoprotein A1 (hnRNP A1), heterogeneous nuclear ribonucleoprotein A2/B1 (hnRNP A2/B1), fused in sarcoma/translocated in liposarcoma (FUS/TLS, herein referred to as FUS), Ewing’s sarcoma breakpoint region 1 (EWSR1), and TAF15 (TATA-box binding protein associated factor 15) (Fig. 1). This evidence concerns the gene HNRNPA1 and amyotrophic lateral sclerosis.