Disruption of axonal mitochondrial transport was also observed in vivo in ALS mutant TDP-43A315T transgenic mice (Magrané et al., 2014) and wild-type TDP-43 and mutant TDP-43M337V overexpressing mice exhibited mitochondrial aggregation consistent with transport defects (Xu et al., 2011, Xu et al., 2010). The gene discussed is TARDBP; the disease is amyotrophic lateral sclerosis.