Recent lines of evidence suggest that in ALS, soluble fractions of TDP-43 interacts with p65 subunit of nuclear factor κB (NF-κB) in the nucleus of neurons and glial cells, and that an upregulation of TDP-43 may contribute to pathogenesis by causing abnormal hyperactivation of p65 NF-κB [23]. This evidence concerns the gene NFKB1 and amyotrophic lateral sclerosis.