Indeed, mutations of mitochondria (i.e., the SOD1 mutant) underpin one of the most widely used (and extensively validated) ALS rodent models (Gurney et al., 1994; Jara et al., 2012; Fogarty, 2018), with mitochondria are a proposed target of recently approved clinical therapies (Takayasu et al., 2007; Writing and Edaravone (MCI-186) ALS 19 Study Group, 2017; Ohta et al., 2020). The gene discussed is SOD1; the disease is amyotrophic lateral sclerosis.