In ALS/FTD models of disease, inhibition of the canonical NF-κB pathway with Withaferin A, an inhibitor of nuclear factor kappa-B kinase subunit gamma (IKK-γ) (also known as NEMO) [86–88] or IMS-088, an analog of Withaferin A, provides therapeutic benefits [69–71]. Here, NFKB1 is linked to amyotrophic lateral sclerosis.