ALS, and TDP-43-mediated neurodegeneration in general, has been associated with not only hyperinflammatory responses, such as nuclear factor κB (NF-κB)-related cytokines (Egawa et al., 2012; Swarup et al., 2011; Zhao et al., 2015) but also with an elevated type I interferon (IFN) signature (Wang et al., 2011). The gene discussed is NFKB1; the disease is amyotrophic lateral sclerosis.