SOD1 and amyotrophic lateral sclerosis: Although CSF concentrations of SOD1 in ALS patients are not known to be significantly different from other individuals (Zetterstrom et al., 2011; Winer et al., 2013), misfolded SOD1 present in CSF obtained from patients with sporadic ALS has been shown to be highly toxic to NSC-34 cells, with antibody-mediated depletion to remove the misfolded SOD1 found to produce a significant reduction in neurotoxicity (Tokuda et al., 2019).