As shown in Figure 1A, TDP-43 contains two RNA recognition motifs that are involved in its function in RNA stabilization and processing, while the carboxy-terminus is believed to drive a toxic gain of function, as the majority of ALS and FTLD-TDP-linked mutations are found in this glycine-rich region of the protein [3], [4], [5], [6]. This evidence concerns the gene TARDBP and amyotrophic lateral sclerosis.