Indeed, although the presence of total TDP43 aggregates was not initially reported in SOD1-related fALS nor in SOD1 mice [46], several recent studies have documented the presence of TDP43 inclusions in motoneurons from SOD1 patients, albeit to a lesser extent compared to other ALS cases [37,47,48]. The gene discussed is TARDBP; the disease is amyotrophic lateral sclerosis.