Phospho-IKKγ (S376) Polyclonal Antibody, 100µg, (ATB-P1043)

Description

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. Multiple transcript variants encoding different isoforms have been found for this gene. A pseudogene highly similar to this locus is located in an adjacent region of the X chromosome. [provided by RefSeq, Aug 2011]

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.

Product datasheet:

References:

1. Correlating interleukin-12 stimulated interferon-γ production and the absence of ectodermal dysplasia and anhidrosis (EDA) in patients with mutations in NF-κB essential modulator (NEMO). Haverkamp MH, et al. J Clin Immunol, 2014 May. PMID 24682681
2. Genomic analysis of a girl with incontinentia pigmenti but without NEMO mutation. Khan AO, et al. Clin Dysmorphol, 2014 Apr. PMID 24487970
3. Insight into IKBKG/NEMO locus: report of new mutations and complex genomic rearrangements leading to incontinentia pigmenti disease. Conte MI, et al. Hum Mutat, 2014 Feb. PMID 24339369
4. Identifying post-translational modifications of NEMO by tandem mass spectrometry after high affinity purification. Jackson SS, et al. Protein Expr Purif, 2013 Nov. PMID 24012789 Free PMC Article
5. Incontinentia pigmenti diagnostic criteria update. Minić S, et al. Clin Genet, 2014 Jun. PMID 23802866
6. Identification of a cell protein (FIP-3) as a modulator of NF-kappaB activity and as a target of an adenovirus inhibitor of tumor necrosis factor alpha-induced apoptosis.
   Li Y., Kang J., Friedman J., Tarassishin L., Ye J., Kovalenko A., Wallach D., Horwitz M.S.
   Proc. Natl. Acad. Sci. U.S.A. 96:1042-1047(1999) [PubMed] [Europe PMC] Cited for: NUCLEOTIDE SEQUENCE [MRNA] (ISOFORM 1).
7. Isolation of full-length cDNA and chromosomal localization of human NF-kappaB modulator NEMO to Xq28.
   Jin D.-Y., Jeang K.-T.
   J. Biomed. Sci. 6:115-120(1999) [PubMed] [Europe PMC] Cited for: NUCLEOTIDE SEQUENCE [MRNA] (ISOFORM 1). Tissue: Mammary cancer.
8. IKK-gamma is an essential regulatory subunit of the IkappaB kinase complex.
   Rothwarf D.M., Zandi E., Natoli G., Karin M.
   Nature 395:297-300(1998) [PubMed] [Europe PMC] Cited for: NUCLEOTIDE SEQUENCE [MRNA] (ISOFORM 1), PARTIAL PROTEIN SEQUENCE. Tissue: Cervix carcinoma.
9. Genomic rearrangement in NEMO impairs NF-kappaB activation and is a cause of incontinentia pigmenti.
   Smahi A., Courtois G., Vabres P., Yamaoka S., Heuertz S., Munnich A., Israel A., Heiss N.S., Klauck S.M., Kioschis P., Wiemann S., Poustka A., Esposito T., Bardaro T., Gianfrancesco F., Ciccodicola A., D’Urso M., Woffendin H.
   , Jakins T., Donnai D., Stewart H., Kenwrick S.J., Aradhya S., Yamagata T., Levy M., Lewis R.A., Nelson D.L.
   Nature 405:466-472(2000) [PubMed] [Europe PMC]

   Cited for: NUCLEOTIDE SEQUENCE [GENOMIC DNA], VARIANT IP VAL-407.

10. cDNA cloning by amplification of circularized first strand cDNAs reveals non-IRE-regulated iron-responsive mRNAs.
    Ye Z., Connor J.R.
    Biochem. Biophys. Res. Commun. 275:223-227(2000) [PubMed] [Europe PMC] Cited for: NUCLEOTIDE SEQUENCE [MRNA] (ISOFORM 1). Tissue: Astrocytoma.