ALS associated mutations are believed to either disrupt these functions, as in the case of VAPB and optineurin, or to confer new functions that are detrimental to mitochondria, as in the case of SOD1 and TDP43 (Cozzolino et al., 2013; Stoica et al., 2014; Wong and Holzbaur, 2014). Here, TARDBP is linked to amyotrophic lateral sclerosis.