TARDBP and amyotrophic lateral sclerosis: In the vast majority of sporadic ALS patients and some familial ALS patients as well, TDP-43 pathology, namely, the disappearance of TDP-43 from the nucleus and the concurrent formation of the inclusion bodies composed of abnormal aggregated TDP-43 in the cytoplasm, has been observed in lower motor neurons, and hence TDP-43 pathology is considered a pathological hallmark of ALS (Arai et al, 2006; Neumann et al, 2006; Sreedharan et al, 2008; Yokoseki et al, 2008; Suk & Rousseaux, 2020).