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NEMONF-kB Essential Modulator. A regulatory sub-unit of the inhibitor of NF-kappaB kinase (IKK) complex encoded by IKBKG on chromosome Xq28, which activates NF-kappaB, triggering activation of genes involved in inflammation, immunity, cell survival, and other pathways. NEMO has an antiapoptotic function independent of its role in the IKK pathway, which prevents receptor-interacting protein-1 (RIP1) from engaging caspase-8 before IKK-mediated induction of antiapoptotic genes. Without NEMO, RIP1 associates with caspase-8, resulting in rapid TNF-induced apoptosis.
IKBKG mutations are linked to incontinentia pigmenti, hypohidrotic ectodermal dysplasia, and several immunodeficiencies.