Neuropathological studies on both ALS patients and mouse models have shown impairment of serotoninergic, dopaminergic, noradrenergic, histaminergic, and cholinergic systems, variably characterized by loss of neurons, altered expression of transporters and receptors, or TDP-43 inclusions [reviewed in Brunet et al. (52)]. This evidence concerns the gene TARDBP and amyotrophic lateral sclerosis.