In 1994, Gurney and colleagues [171] developed transgenic mice that overexpressed mutant SOD1 (mSOD1) and were characterized by progressive motoneuron degeneration, which recapitulated several pathophysiological hallmarks of ALS, including cytoplasmic mislocalization of TAR DNA-binding protein 43 (TDP-43) at the end-stage of the disease [172]. The gene discussed is TARDBP; the disease is amyotrophic lateral sclerosis.