Mouse IKBKG Monoclonal Antibody | anti-IKBKG antibody
IKBKG (Inhibitor of kappa Light Polypeptide Gene Enhancer in B-Cells, Kinase gamma, AMCBX1, FIP-3, FIP3, Fip3p, IKK-gamma, IP, IP1, IP2, IPD2, NEMO) (MaxLight 405)
NCBI and Uniprot Product Information
NCBI Description
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. [provided by RefSeq, Mar 2016]
Uniprot Description
IKKG: a regulatory subunit of the IKK-signalosome complex. Interacts preferentially with IKK-beta but also able to interact with IKK-alpha, IKAP, TAX, RIP and MAP3K14/NIK. Defects are the cause of familial incontinentia pigmenti type II (IP2).
Protein type: Protein kinase, regulatory subunit; Adaptor/scaffold
Chromosomal Location of Human Ortholog: Xq28
Cellular Component: spindle pole; cytoplasm; intracellular; IkappaB kinase complex; nucleus; cytosol; ubiquitin ligase complex
Molecular Function: protein domain specific binding; signal transducer activity; peroxisome proliferator activated receptor binding; protein binding; protein homodimerization activity; protein heterodimerization activity; metal ion binding; ubiquitin protein ligase binding
Biological Process: I-kappaB kinase/NF-kappaB cascade; establishment of vesicle localization; viral reproduction; activation of MAPK activity; apoptosis; stress-activated MAPK cascade; toll-like receptor 3 signaling pathway; T cell receptor signaling pathway; activation of NF-kappaB transcription factor; toll-like receptor 10 signaling pathway; toll-like receptor 5 signaling pathway; B cell homeostasis; positive regulation of interferon type I production; JNK cascade; inflammatory response; toll-like receptor 4 signaling pathway; positive regulation of I-kappaB kinase/NF-kappaB cascade; transcription, DNA-dependent; MyD88-independent toll-like receptor signaling pathway; response to virus; activation of NF-kappaB-inducing kinase; toll-like receptor 2 signaling pathway; MyD88-dependent toll-like receptor signaling pathway; toll-like receptor signaling pathway; innate immune response; positive regulation of transcription from RNA polymerase II promoter; immune response; toll-like receptor 9 signaling pathway; response to DNA damage stimulus
Disease: Invasive Pneumococcal Disease, Recurrent Isolated, 2; Ectodermal Dysplasia, Hypohidrotic, With Immune Deficiency; Immunodeficiency Without Anhidrotic Ectodermal Dysplasia; Immunodeficiency 33; Incontinentia Pigmenti; Ectodermal Dysplasia, Anhidrotic, With Immunodeficiency, Osteopetrosis, And Lymphedema