In vitro as well as in vivo models of ALS, which have been established in order to investigate the consequences upon expression of mutant SOD1, TDP-43 and FUS, respectively, revealed both aggregation and fragmentation of mitochondria (Dal Canto and Gurney 1994; Higgins et al., 2003; De Vos et al., 2007; Vande Velde et al., 2011; Hong et al., 2012; Wang W et al., 2013; Magrane et al., 2014), suggesting impaired mitochondrial dynamics to be a disease-contributing factor. Here, TARDBP is linked to amyotrophic lateral sclerosis.