TARDBP and amyotrophic lateral sclerosis: It has also been previously shown that human motor neurons with transactive response DNA-binding protein 43 (TDP-43) pathological mislocalization have increased AMPK activation; in addition, SOD1-ALS mouse models have revealed the upregulation of the AMPK-mediated phosphorylation of compounds linked to autophagy in the spinal cord [12,20].