The pathology of sporadic amyotrophic lateral sclerosis (sALS) is associated with the dysregulation of the 43-kDa transactive response DNA-binding protein (TDP-43) [3, 4, 37, 49, 54, 66] that leads to the formation of proteinaceous cytoplasmic aggregates in specific cortical and subcortical projection neurons with long axons, whereas cells with short axons are spared [27, 60]. This evidence concerns the gene TARDBP and sporadic amyotrophic lateral sclerosis.