SOD1 and amyotrophic lateral sclerosis: In SOD1 or TDP-43-associated familial ALS mitochondrial damage appears to be directly linked to pathological accumulations of aggregated ALS mutant SOD1 or TDP-43 in mitochondria (Magrané et al., 2009, Igoudjil et al., 2011, Pickles et al., 2013, Pickles et al., 2016, Israelson et al., 2010, Li et al., 2010, Pasinelli et al., 2004, Liu et al., 2004, Cozzolino et al., 2009, Wang et al., 2016).