TDP-43, encoded by the TARDBP gene, is the major aggregated protein involved in the formation of the characteristic inclusions, especially in its hyperphosphorylated and ubiquitin-bound form, in the brains of patients with ubiquitin-positive frontotemporal lobar degeneration (FTLD-U) and or ALS [141]. This evidence concerns the gene TARDBP and amyotrophic lateral sclerosis.