|Other Names||NF-kappa-B essential modulator, NEMO, 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, IKBKG, FIP3, NEMO|
|Format||Synthetic peptide was lyophilized with 100% acetonitrile and is supplied as a powder. Reconstitute with 0.1 ml DI water for a final concentration of 1 mg/ml.|
|Storage||Maintain refrigerated at 2-8°C for up to 6 months. For long term storage store at -20°C.|
|Precautions||This product is for research use only. Not for use in diagnostic or therapeutic procedures.|
|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.|
|Cellular Location||Cytoplasm. Nucleus. Note=Sumoylated NEMO accumulates in the nucleus in response to genotoxic stress|
|Tissue Location||Heart, brain, placenta, lung, liver, skeletal muscle, kidney and pancreas|
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This gene encodes the regulatory subunit of the inhibitorof kappaB kinase (IKK) complex, which activates NF-kappaB resultingin activation of genes involved in inflammation, immunity, cellsurvival, and other pathways. Mutations in this gene result inincontinentia pigmenti, hypohidrotic ectodermal dysplasia, andseveral other types of immunodeficiencies. Multiple transcriptvariants encoding different isoforms have been found for this gene.A pseudogene highly similar to this locus is located in an adjacentregion of the X chromosome.
Boehm, D., et al. Proc. Natl. Acad. Sci. U.S.A. 107(42):18103-18108(2010)Wu, Z.H., et al. Mol. Cell 40(1):75-86(2010)Arimoto, K., et al. Proc. Natl. Acad. Sci. U.S.A. 107(36):15856-15861(2010)Gautheron, J., et al. Hum. Mol. Genet. 19(16):3138-3149(2010)Mooster, J.L., et al. J. Allergy Clin. Immunol. 126(1):127-132(2010)
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