Despite a consensus that TDP-43 pathology is absent in SOD1-ALS (Mackenzie et al., 2007), cytoplasmic TDP-43 aggregates were found in spinal motor neurons in old mice and SOD1G93A, showing a stark overlap in pathology between ageing and ALS (Valdez et al., 2012). This evidence concerns the gene SOD1 and amyotrophic lateral sclerosis.