Amongst these, neuronal and glial cytoplasmic inclusions immunoreactive for TDP-43 are observed in virtually (approaching 97%) all ALS patients, except superoxide dismutase 1 (SOD1) and FUS/TLS cases (Andersen et al., 1995; Al-Sarraj et al., 2011; Boxer et al., 2011; Couthouis et al., 2011; Deng et al., 2011; Hortobagyi et al., 2011; Couthouis et al., 2012). The gene discussed is SOD1; the disease is amyotrophic lateral sclerosis.