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Mouse Anti-IKBKG Recombinant Antibody (55AT986.5.78) (CBMAB-I1394-YY)

This product is Mouse antibody that recognizes IKBKG . The antibody 55AT986.5.78 can be used for immunoassay techniques such as: ELISA, WB
See all IKBKG antibodies

Summary

Host Animal
Mouse
Specificity
Human
Clone
55AT986.5.78
Antibody Isotype
IgG1
Application
ELISA, WB

Basic Information

Specificity
Human
Antibody Isotype
IgG1
Application Notes
The COA includes recommended starting dilutions, optimal dilutions should be determined by the end user.

Formulations & Storage [For reference only, actual COA shall prevail!]

Format
Liquid
Buffer
PBS, pH 7.2
Preservative
0.09% sodium azide
Storage
Store at +4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freeze/thaw cycles.

Target

Full Name
Inhibitor Of Nuclear Factor Kappa B Kinase Subunit Gamma
Introduction
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.
Entrez Gene ID
8517
UniProt ID
Q9Y6K9
Alternative Names
Inhibitor Of Nuclear Factor Kappa B Kinase Subunit Gamma
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 (PubMed:9751060, PubMed:14695475, PubMed:20724660).

Its binding to scaffolding polyubiquitin plays a key role in IKK activation by multiple signaling receptor pathways (PubMed:16547522, PubMed:18287044, PubMed:19033441, PubMed:21606507, PubMed:27777308, PubMed:19185524, PubMed:33567255).

Can recognize and bind both 'Lys-63'-linked and linear polyubiquitin upon cell stimulation, with a much higher affinity for linear polyubiquitin (PubMed:16547522, PubMed:18287044, PubMed:27777308, PubMed:19033441, PubMed:21606507, PubMed:19185524).

Could be implicated in NF-kappa-B-mediated protection from cytokine toxicity. Essential for viral activation of IRF3 (PubMed:19854139).

Involved in TLR3- and IFIH1-mediated antiviral innate response; this function requires 'Lys-27'-linked polyubiquitination (PubMed:20724660).

(Microbial infection) Also considered to be a mediator for HTLV-1 Tax oncoprotein activation of NF-kappa-B.
Biological Process
Anoikis Source: BHF-UCL
Apoptotic process Source: ProtInc
Cellular response to DNA damage stimulus Source: UniProtKB
Establishment of vesicle localization Source: UniProtKB
I-kappaB kinase/NF-kappaB signaling Source: UniProtKB
Immune response Source: ProtInc
Inflammatory response Source: UniProtKB
Innate immune response Source: UniProtKB
Negative regulation of neuron death Source: ParkinsonsUK-UCL
Positive regulation of I-kappaB kinase/NF-kappaB signaling Source: UniProtKB
Positive regulation of macroautophagy Source: BHF-UCL
Positive regulation of NF-kappaB transcription factor activity Source: UniProtKB
Positive regulation of T cell receptor signaling pathway Source: UniProtKB
Positive regulation of transcription by RNA polymerase II Source: UniProtKB
Protein-containing complex assembly Source: UniProtKB
Regulation of I-kappaB kinase/NF-kappaB signaling Source: GO_Central
Response to virus Source: UniProtKB
T cell receptor signaling pathway Source: UniProtKB
Cellular Location
Nucleus; Cytoplasm. Sumoylated NEMO accumulates in the nucleus in response to genotoxic stress.
Involvement in disease
Ectodermal dysplasia and immunodeficiency 1 (EDAID1):
A form of ectoderma dysplasia, a heterogeneous group of disorders due to abnormal development of two or more ectodermal structures. EDAID1 is an X-linked recessive disorder characterized by absence of sweat glands, sparse scalp hair, rare conical teeth and immunological abnormalities resulting in severe infectious diseases. Severely affected individuals may also show lymphedema, osteopetrosis, and, rarely, hematologic abnormalities. The phenotype is highly variable, and may be fatal in childhood.
Immunodeficiency 33 (IMD33):
An X-linked recessive disorder characterized by variably impaired immunologic function and early-onset recurrent infections, usually due to pneumococcus, H. influenzae, and atypical mycobacteria. Features of hypohidrotic ectodermal dysplasia are generally not present, although some patients may have conical teeth or hypodontia.
Incontinentia pigmenti (IP):
A genodermatosis usually prenatally lethal in males. In affected females, it causes abnormalities of the skin, hair, eyes, nails, teeth, skeleton, heart, and central nervous system. The prominent skin signs occur in four classic cutaneous stages: perinatal inflammatory vesicles, verrucous patches, a distinctive pattern of hyperpigmentation and dermal scarring.
PTM
Phosphorylation at Ser-68 attenuates aminoterminal homodimerization.
Polyubiquitinated on Lys-285 through 'Lys-63'; the ubiquitination is mediated by NOD2 and RIPK2 and probably plays a role in signaling by facilitating interactions with ubiquitin domain-containing proteins and activates the NF-kappa-B pathway. Polyubiquitinated on Lys-399 through 'Lys-63'; the ubiquitination is mediated by BCL10, MALT1 and TRAF6 and probably plays a role in signaling by facilitating interactions with ubiquitin domain-containing proteins and activates the NF-kappa-B pathway. Monoubiquitinated on Lys-277 and Lys-309; promotes nuclear export. Polyubiquitinated through 'Lys-27' by TRIM23; involved in antiviral innate and inflammatory responses. Linear polyubiquitinated on Lys-111, Lys-143, Lys-226, Lys-246, Lys-264, Lys-277, Lys-285, Lys-292, Lys-302, Lys-309 and Lys-326; the head-to-tail polyubiquitination is mediated by the LUBAC complex and plays a key role in NF-kappa-B activation. Deubiquitinated by USP10 in a TANK-dependent and -independent manner, leading to the negative regulation of NF-kappa-B signaling upon DNA damage (PubMed:25861989). Ubiquitinated at Lys-326 by MARCHF2 following bacterial and viral infection which leads to its degradation (PubMed:32935379).
Sumoylated on Lys-277 and Lys-309 with SUMO1; the modification results in phosphorylation of Ser-85 by ATM leading to a replacement of the sumoylation by mono-ubiquitination on these residues.
Neddylated by TRIM40, resulting in stabilization of NFKBIA and down-regulation of NF-kappa-B activity.
(Microbial infection) Cleaved by hepatitis A virus (HAV) protease 3C allowing the virus to disrupt the host innate immune signaling.
(Microbial infection) Polyubiquitinated on Lys-309 and Lys-321 via 'Lys-27'-linked ubiquitin by Shigella flexneri E3 ubiquitin-protein ligase ipah9.8, leading to its degradation by the proteasome.
(Microbial infection) Polyubiquitination through 'Lys-63' is interrupted by interaction with SARS coronavirus-2/SARS-CoV-2 virus protein ORF9B which inhibits the NF-kappa-B pathway.

Zhang, Z., Pan, Y., Zhao, Y., Ren, M., Li, Y., Lu, G., & He, S. (2023). RIPK2 promotes the progression of colon cancer by regulating BIRC3-mediated ubiquitination of IKBKG. Experimental Cell Research, 429(1), 113644.

Minić, S., Cerovac, N., Novaković, I., Gazikalović, S., Popadić, S., & Trpinac, D. (2023). The Impact of the IKBKG Gene on the Appearance of the Corpus Callosum Abnormalities in Incontinentia Pigmenti. Diagnostics, 13(7), 1300.

Wu, C. X., Mao, C. Y., Deng, J., Zhang, T., Zhao, Y., Guan, Z. Z., ... & Qi, X. L. (2022). Fluoride induced down-regulation of IKBKG Gene expression inhibits hepatocytes senescence. Journal of Trace Elements in Medicine and Biology, 69, 126896.

Kawai, M., Sugimoto, A., Ishihara, Y., Kato, T., & Kurahashi, H. (2022). Incontinentia pigmenti inherited from a father with a low level atypical IKBKG deletion mosaicism: a case report. BMC pediatrics, 22(1), 378.

Haque, M. N., Ohtsubo, M., Nishina, S., Nakao, S., Yoshida, K., Hosono, K., ... & Minoshima, S. (2021). Analysis of IKBKG/NEMO gene in five Japanese cases of incontinentia pigmenti with retinopathy: fine genomic assay of a rare male case with mosaicism. Journal of Human Genetics, 66(2), 205-214.

Gao, S., Menendez, M., Kurylowicz, K., & Griffin, C. T. (2021). Genomic locus proteomic screening identifies the NF-κB signaling pathway components NFκB1 and IKBKG as transcriptional regulators of Ripk3 in endothelial cells. PloS one, 16(6), e0253519.

Kawai, M., Kato, T., Tsutsumi, M., Shinkai, Y., Inagaki, H., & Kurahashi, H. (2020). Molecular analysis of low‐level mosaicism of the IKBKG mutation using the X Chromosome Inactivation pattern in Incontinentia Pigmenti. Molecular genetics & genomic medicine, 8(12), e1531.

Moeng, S., Seo, H. A., Hwang, C. Y., Cipolla, G. A., Lee, D. J., Kuh, H. J., & Park, J. K. (2018). MicroRNA-107 targets IKBKG and sensitizes A549 cells to parthenolide. Anticancer Research, 38(11), 6309-6316.

Hsu, A. P., Zerbe, C. S., Foruraghi, L., Iovine, N. M., Leiding, J. W., Mushatt, D. M., ... & Holland, S. M. (2018). IKBKG (NEMO) 5′ untranslated splice mutations lead to severe, chronic disseminated mycobacterial infections. Clinical Infectious Diseases, 67(3), 456-459.

Frans, G., Meert, W., Ten Bosch, J. V. D. W., Meyts, I., Bossuyt, X., Vermeesch, J. R., & Hestand, M. S. (2018). Conventional and single-molecule targeted sequencing method for specific variant detection in IKBKG while bypassing the IKBKGP1 pseudogene. The Journal of Molecular Diagnostics, 20(2), 195-202.

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For research use only. Not intended for any clinical use.

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