Reductions in energy metabolism have been reported in ALS (Vandoorne et al., 2018) and mitochondrial abnormalities, such as swelling and morphological changes, are among the earliest signs of pathology in SOD1G93A and SOD1G37R mice (Wong et al., 1995; Kong and Xu, 1998), FUSR521C rats (Huang et al., 2012; So et al., 2018) and wild type TDP-43 mice (Shan et al., 2010; Xu et al., 2010). Here, TARDBP is linked to amyotrophic lateral sclerosis.