Although the majority of ubiquitinated inclusions are immunoreactive for TDP-43, notable exceptions are the inclusions found in ALS patients with mutations in SOD1 or FUS, which are negative for ubiquitinated TDP-43 but immunoreactive for mutant aggregated Cu/Zn superoxide dismutase (SOD1) and fused in sarcoma protein (FUS) respectively (Watanabe et al., 2001; Wang et al., 2002; Mackenzie et al., 2007; Kwiatkowski et al., 2009; Vance et al., 2009). This evidence concerns the gene SOD1 and amyotrophic lateral sclerosis.