NF-kappa-B essential modulator; NEMO; NF-kappa-B essential modifier; inhibitor of nuclear factor kappa-B kinase subunit gamma; IkB kinase subunit gamma; I-kappa-B kinase gamma; IKK-gamma; IKKG; IkB kinase-associated protein 1; IKKAP1;FIP-3 (IKBKG, FIP3, NEMO)
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Function
-regulatory subunit of the IKK core complex
- does not have kinase activity
- also considered to be a mediator for TAX activation of NF-kappa-B
- could be implicated in NF-kappa B mediated protection from cytokine toxicity (putative)
- phosphorylation at Ser-68 attenuates aminoterminal homodimerization
- polyubiquitinated on Lys-285 through Lys-63; ubiquitination is mediated by NOD2 & RIPK2
- polyubiquitinated on Lys-399 through Lys-63; ubiquitination is mediated by BCL10, MALT1 & TRAF6
- ubiquitination probably plays a role in signaling by facilitating interactions with ubiquitin domain-containing proteins
- ubiquitination activates the NF-kappa-B pathway
- sumoylated on Lys-277 & Lys-309 by SUMO1; this results in phosphorylation of Ser-85 by ATM leading to a replacement of the sumoylation by mono-ubiquitination on these residues
- mono-ubiquitinated on Lys-277 & Lys-309; promotes nuclear export
- part of a complex composed of NCOA2, NCOA3, CHUK/IKKA, IKBKB, IKBKG & CREBBP
- interacts with COPS3, CYLD, NALP2, TRPC4AP & LRDD
- interacts with ATM; the complex is exported from the nucleus
- interacts with TRAF6
- interacts with HTLV-1 Tax oncoprotein; the interaction activates IKBKG
- interacts with TANK; the interaction is enhanced by IKBKE & TBK1
- part of a ternary complex consisting of TANK, IKBKB & IKBKG
Structure
- homodimer; disulfide-linked
- contains 1 C2HC-type Zn+2 finger
Compartment
Expression
- expressed in heart, brain, placenta, lung, liver, skeletal muscle, kidney & pancreas
Pathology
- defects in IKBKG are the cause of
- X-linked anhidrotic ectodermal dysplasia with immunodeficiency
- anhidrotic ectodermal dysplasia with immunodeficiency- osteopetrosis-lymphedema
- isolated immunodeficiency (without ectodermal dysplasia)
- X-linked susceptibility to mycobacterial disease type 1
- recurrent isolated invasive pneumococcal disease type 2
- incontinentia pigmenti
More general terms
Component of
References
- ↑ Rothwarf DM et al IKK-gamma is an essential regulatory subunit of the IkappaB kinase complex. Nature 395:297-300, 1998 PMID: https://www.ncbi.nlm.nih.gov/pubmed/9751060
- ↑ Yamaoka S et al Complementation cloning of NEMO, a component of the IkappaB kinase complex essential for NF-kappaB activation. Cell 93:1231-40, 1998 PMID: https://www.ncbi.nlm.nih.gov/pubmed/9657155
- ↑ UniProt http://www.uniprot.org/uniprot/Q9Y6K9.html
- ↑ IKBKGbase; Note: IKBKG mutation db http://bioinf.uta.fi/IKBKGbase/
- ↑ GeneReviews https://www.genecards.org/cgi-bin/carddisp.pl?gene=IKBKG
Database
- Entrez gene: http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&cmd=Retrieve&dopt=Graphics&list_uids=8517
- Kegg: http://www.genome.jp/dbget-bin/www_bget?hsa:8517
- OMIM: https://mirror.omim.org/entry/300248
- OMIM: https://mirror.omim.org/entry/300291
- OMIM: https://mirror.omim.org/entry/300301
- OMIM: https://mirror.omim.org/entry/300584
- OMIM: https://mirror.omim.org/entry/300636
- OMIM: https://mirror.omim.org/entry/300640
- OMIM: https://mirror.omim.org/entry/308300
- UniProt: http://www.uniprot.org/uniprot/Q9Y6K9.html