Nevertheless, the importance of TDP-43 in ALS relies on the fact that it is a major component of the ubiquitinated insoluble cytoplasmic inclusions, concomitant with a loss of nuclear TDP-43 in upper and lower motor neurons and in other regions of the central nervous system in most patients (both sporadic and familial, with or without TARDBP mutations) [10]. The gene discussed is TARDBP; the disease is amyotrophic lateral sclerosis.