ALS patients with mutations in superoxide dismutase 1 (SOD1) or RNA-binding protein FUS (FUS) are negative for ubiquitinated inclusions of TDP-43 but immunoreactive for mutant aggregated Cu/Zn SOD1 and fused in sarcoma protein (FUS), respectively [65,66]. Here, SOD1 is linked to amyotrophic lateral sclerosis.