IKK gamma (IKBKG, FIP3, NEMO, NF-kappa-B essential modulator, FIP-3, IkB kinase-associated protein 1, Inhibitor of nuclear factor kappa-B kinase subunit gamma, NF-kappa-B essential modifier) (FITC)

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.\n\nApplications:\nSuitable for use in Western Blot, ELISA.\n\nRecommended Dilution:\nELISA: 1:1,000\nWestern Blot: 1:100~500\n\nStorage and Stability:\nMay be stored at 4 degrees C for short-term only. For long-term storage, store at -20 degrees C. Aliquots are stable for at least 6 months at -20 degrees C. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap. Further dilutions can be made in assay buffer.

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SPECIFICATIONS

Catalog Number

031047-FITC

Size

100ul

Applications

ELISA, WB

Hosts

Mouse

Reactivities

Hum

Form

Supplied as a liquid in PBS, pH 7.2. Contains ~0.09% sodium azide. Conjugated to FITC.

P Type

Mab

Purity

Purified by Protein G affinity chromatography.

Isotype

IgG1

References

Immune deficiency caused by impaired expression of nuclear factor-kappaB essential modifier (NEMO) because of a mutation in the 5' untranslated region of the NEMO gene. Mooster JL, et al. J Allergy Clin Immunol, 2010 Jul. PMID 20542322.\nNEMO gene mutations in Chinese patients with incontinentia pigmenti. Hsiao PF, et al. J Formos Med Assoc, 2010 Mar. PMID 20434027.\nThe LCR at the IKBKG locus is prone to recombine. Fusco F, et al. Am J Hum Genet, 2010 Apr 9. PMID 20380930.\nIKK{gamma} protein is a target of BAG3 regulatory activity in human tumor growth. Ammirante M, et al. Proc Natl Acad Sci U S A, 2010 Apr 20. PMID 20368414.\nActivation of noncanonical NF-kappaB signaling by the oncoprotein Tio. de Jong SJ, et al. J Biol Chem, 2010 May 28. PMID 20353939.

Additional Info

Human

Alternative Names

IKBKG, FIP3, NEMO, NF-kappa-B essential modulator, FIP-3, IkB kinase-associated protein 1, Inhibitor of nuclear factor kappa-B kinase subunit gamma, NF-kappa-B essential modifier