IKBKG (inhibitor of kappa light polypeptide gene enhancer in B-cells, kinase gamma) encodes the regulatory subunit of the inhibitor of kappaB kinase (IKK) complex, which activates NF-kappaB resulting in activation of genes involved in inflammation, immunity, cell survival, and other pathways. Mutations in IKBKG result in incontinentia pigmenti, hypohidrotic ectodermal dysplasia, and several other types of immunodeficiencies. A pseudogene highly similar to IKBKG is located in an adjacent region of the X chromosome.