Synonyms: | IKBKG, AMCBX1, FIP-3, FIP3, Fip3p, IKK-gamma, IKKAP1, IKKG, IMD33, IP, IP1, IP2, IPD2, NEMO, ZC2HC9 |
Background: | 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. |
UniProt Protein Function: | Regulatory subunit of the IKK core complex which phosphorylates inhibitors of NF-kappa-B thus leading to the dissociation of the inhibitor/NF-kappa-B complex and ultimately the degradation of the inhibitor. Its binding to scaffolding polyubiquitin seems to play a role in IKK activation by multiple signaling receptor pathways. However, the specific type of polyubiquitin recognized upon cell stimulation (either 'Lys-63'-linked or linear polyubiquitin) and its functional importance is reported conflictingly. 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. Essential for viral activation of IRF3. Involved in TLR3- and IFIH1-mediated antiviral innate response; this function requires 'Lys-27'-linked polyubiquitination. |
NCBI Summary: | 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. [provided by RefSeq, Mar 2016] |
UniProt Code: | Q9Y6K9 |
NCBI GenInfo Identifier: | 6685695 |
NCBI Gene ID: | 8517 |
NCBI Accession: | Q9Y6K9.2 |
UniProt Secondary Accession: | Q9Y6K9,Q7LBY6, Q7Z7F1, |
UniProt Related Accession: | Q9Y6K9 |
Molecular Weight: | 36,953 Da |
NCBI Full Name: | NF-kappa-B essential modulator |
NCBI Synonym Full Names: | inhibitor of nuclear factor kappa B kinase subunit gamma |
NCBI Official Symbol: | IKBKG |
NCBI Official Synonym Symbols: | IP; IP1; IP2; FIP3; IKKG; IPD2; NEMO; FIP-3; Fip3p; IMD33; AMCBX1; IKKAP1; ZC2HC9; IKK-gamma |
NCBI Protein Information: | NF-kappa-B essential modulator |
UniProt Protein Name: | NF-kappa-B essential modulator |
UniProt Synonym Protein Names: | FIP-3; IkB kinase-associated protein 1; IKKAP1; Inhibitor of nuclear factor kappa-B kinase subunit gamma; I-kappa-B kinase subunit gamma; IKK-gamma; IKKG; IkB kinase subunit gamma; NF-kappa-B essential modifier |
UniProt Gene Name: | IKBKG |