2JVX, 2JVY, 3BRT, 3BRV, 3CL3, 3FX0, 4BWN, 5AAY851716151ENSG00000269335ENSMUSG00000004221Q9Y6K9O88522NM_001321397NM_001377312NM_001377313NM_001377314NM_001377315NM_010547NM_178590NP_003630NP_001364241NP_001364242NP_001364243NP_001364244NP_034677NP_848705NF-kappa-B essential modulator (NEMO) also known as inhibitor of nuclear factor kappa-B kinase subunit gamma (IKK-γ) is a protein that in humans is encoded by the IKBKG gene.

Mutations in the IKBKG gene results in incontinentia pigmenti,[7] hypohidrotic ectodermal dysplasia,[8] and several other types of immunodeficiencies.

An increased level of NEMO gene expression was found in the blood of pregnant women with preeclampsia and their children.

[9] However, a decrease of the mRNA levels of total NEMO and the transcripts 1A, 1B, and 1C in placentas derived from preeclamptic women may be the main reason for intensified apoptosis.

In the absence of regulatory subunit IKK-γ the IKK complex is inactive, preventing the downstream signal transduction cascade leading to NF-κB activation.

[12] A small region on the carboxyl terminus of IKK-α (L738-L743) and IKK-β (L737-L742) is essential for a stable interaction with NEMO and for the assembly of the active IKK complex.

The NBD peptide consists of the region from T735 to E745 of the IKK-β subunit fused with a sequence derived from the Antennapedia homeodomain that mediates membrane translocation.

Furthermore, wild type NBD peptide has been shown to dose-dependently inhibit interaction of IKKB with NEMO compared to mutant controls.