Three years after the discovery of TDP-43 as the major component of inclusions in the central nervous system of nearly all ALS patients, FUS was identified as the primary component of TDP-43-negative cytoplasmic inclusions in rare patients with ALS, all of whom had causative genetic mutations in FUS (Vance et al., 2009; Suzuki et al., 2010; Tateishi et al., 2010). This evidence concerns the gene TARDBP and amyotrophic lateral sclerosis.