While activation of NF-κB, the upstream effector of iNOS, is well established in animal models and human ALS (Casciati et al. 2002; Migheli et al. 1997; Sako et al. 2012; Xu et al. 2018), the involvement of the various cellular components of NF-κB activation and its role in disease is ambiguous. This evidence concerns the gene NFKB1 and amyotrophic lateral sclerosis.