
This gene 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 this gene result in incontinentia pigmenti| hypohidrotic ectodermal dysplasia| and several other types of immunodeficiencies. A pseudogene highly similar to this locus is located in an adjacent region of the X chromosome.

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