Many ALS mutants show a loss of OPTN-mediated NF-κB suppression (Nakazawa et al., 2016), deficiencies in OPTN expression increase NF-κB activity and susceptibility to PDB (Obaid et al., 2015) and a subset of CD patients with reduced OPTN expression display impaired secretion of TNF-α, IL6 and IFN-γ (Smith et al., 2015). Here, NFKB1 is linked to amyotrophic lateral sclerosis.