The following IKBKG reagents supplied by CUSABIO are manufactured under a strict quality control system. Multiple applications have been validated and solid technical support is offered.

IKBKG Antibodies

IKBKG Antibodies for Homo sapiens (Human)

IKBKG Proteins

IKBKG Proteins for Mus musculus (Mouse)

IKBKG Proteins for Rattus norvegicus (Rat)

IKBKG Proteins for Bos taurus (Bovine)

IKBKG Background

Inhibitory kappa-B kinase gamma (IKKγ) is a 48 kDa non-catalytic protein in humans that is encoded by the IKBKG gene. IKKγ, also known as nuclear factor kappa B (NF-κB) essential modulator (NEMO), is a subunit of the inhibitory kappa-B (IκB) kinase complex that activates NF-κB leading to activation of genes involved in inflammation, immunity, cell survival, and other pathways against apoptosis. Molecular cloning and sequencing indicate that IKKγ is composed of several potential coiled-coil motifs. It plays a scaffolding/regulatory role and is required for kinase function [1][2]. IKKγ interacts preferentially with IKKβ and is indispensable for the activation of the IKK complex [3]. Binding of IKKγ to IKKα and IKKβ subunits activate the IKK complex leading to phosphorylation of IκB kinase IκBα and the release of NF-κB dimers p105 and RELA to translocate to the nucleus and activate transcription of NF-κB responsive genes. An IKKγ C-terminal truncation mutant that still binds IKKβ blocks the activation of IKK and NF-κB [3]. An N-terminal α-helical region of IKKγ associated with a C-terminal segment of IKKα and IKKβ is termed the NEMO-binding domain (NBD). A cell-permeable NBD peptide blocked association of NEMO with the IKK complex and inhibited cytokine-induced NF-κB activation and NF-κB–dependent gene expression [4]. Lack of IKKγ, therefore, makes cells more prone to apoptosis. Hypomorphic mutations in IKBKG lead to X-linked anhidrotic ectodermal dysplasia with immunodeficiency or to isolated immunodeficiency. IKBKG mutations also result in incontinentia pigmenti [5], hypohidrotic ectodermal dysplasia [6], and several other types of immunodeficiencies.

[1] Rothwarf DM, Zandi E, et al. IKK-gamma is an essential regulatory subunit of the IkappaB kinase complex [J]. Nature. 1998 Sep 17; 395(6699):297-300.
[2] Yamaoka S, Courtois G, et al. Complementation cloning of NEMO, a component of the IkappaB kinase complex essential for NF-kappaB activation [J]. Cell. 1998 Jun 26; 93(7):1231-40.
[3] David M. Rothwarf, Ebrahim Zandi, et al. IKK-γ is an essential regulatory subunit of the IκB kinase complex [J]. Nature volume 395, pages297-300(1998).
[4] Michael J. May, Fulvio D'Acquisto, et al. Selective Inhibition of NF-κB Activation by a Peptide That Blocks the Interaction of NEMO with the IκB Kinase Complex [J]. Science. 2000:Vol. 289, Issue 5484, pp. 1550-1554.
[5] Aradhya S, Woffendin H, et al. A recurrent deletion in the ubiquitously expressed NEMO (IKK-gamma) gene accounts for the vast majority of incontinentia pigmenti mutations [J]. Human Molecular Genetics. 2001, 10 (19): 2171-9.
[6] Zonana J, Elder ME, et al. A novel X-linked disorder of immune deficiency and hypohidrotic ectodermal dysplasia is allelic to incontinentia pigmenti and due to mutations in IKK-gamma (NEMO) [J]. American Journal of Human Genetics. 2000, 67 (6): 1555-62.

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