TAR DNA binding protein-43 (TDP-43) is the major component of these inclusions (Arai et al., 2006; Neumann et al., 2006) in almost all (∼97%) ALS patients and ∼50% FTD patients (Arai et al., 2006; Neumann et al., 2006; Mackenzie et al., 2007; Scotter et al., 2015; Le et al., 2016). This evidence concerns the gene TARDBP and amyotrophic lateral sclerosis.