OPTN and amyotrophic lateral sclerosis: Several ALS-linked proteins were found to modulate the NF-κB pathway: TDP-43 upregulation can enhance NF-κB activation [14], mutation in valosin-containing protein (VCP) in mice resulted in NF-κB hyperactivation [35, 36] and suppression of Optineurin (OPTN) led to neuronal death via NF-κB pathway [37].