Abnormally aggregated mitochondria, as seen in mouse models for TDP-43 and FUS/TLS-mediated ALS, suggest for deficits in mitochondrial trafficking in motor neurons, a phenomenon which has already been proven in mouse models expressing ALS-associated mutant SOD1 (Martin, 2011; Cozzolino et al., 2012). This evidence concerns the gene TARDBP and amyotrophic lateral sclerosis.