Summary: 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].

inhibitor of nuclear factor kappa B kinase subunit gammaMIM:300248Ensembl:ENSG00000269335HGNC:HGNC:5961PA29777Xq28

GO terms in IKBKG

Term TypeEvidence TypeGO Term IDGO Des.
MFIPIGO:0005515protein binding
MFIPIGO:0019904protein domain specific binding
MFIPIGO:0031625ubiquitin protein ligase binding
MFIPIGO:0042802identical protein binding
MFIDAGO:0042803protein homodimerization activity
MFIEAGO:0042975peroxisome proliferator activated receptor binding
MFIEAGO:0046872metal ion binding
MFIDAGO:0046982protein heterodimerization activity
MFIBAGO:0070530K63-linked polyubiquitin modification-dependent protein binding
MFIDAGO:1990450linear polyubiquitin binding
CCIPIGO:0000151ubiquitin ligase complex
CCIDAGO:0000922spindle pole
CCIBAGO:0008385IkappaB kinase complex
CCIDAGO:0008385IkappaB kinase complex
CCTASGO:0008385IkappaB kinase complex
CCTASGO:0008385IkappaB kinase complex
CCIDAGO:0072686mitotic spindle
BPTASGO:0000187activation of MAPK activity
BPTASGO:0002223stimulatory C-type lectin receptor signaling pathway
BPTASGO:0002479antigen processing and presentation of exogenous peptide antigen via MHC class I, TAP-dependent
BPTASGO:0002756MyD88-independent toll-like receptor signaling pathway
BPIEAGO:0006351transcription, DNA-templated
BPTASGO:0006915apoptotic process
BPTASGO:0006954inflammatory response
BPTASGO:0006955immune response
BPIDAGO:0006974cellular response to DNA damage stimulus
BPIMPGO:0006974cellular response to DNA damage stimulus
BPTASGO:0007249I-kappaB kinase/NF-kappaB signaling
BPTASGO:0007254JNK cascade
BPTASGO:0009615response to virus
BPTASGO:0010803regulation of tumor necrosis factor-mediated signaling pathway
BPIEAGO:0016032viral process
BPISSGO:0016239positive regulation of macroautophagy
BPTASGO:0016579protein deubiquitination
BPTASGO:0035666TRIF-dependent toll-like receptor signaling pathway
BPTASGO:0038095Fc-epsilon receptor signaling pathway
BPIBAGO:0043122regulation of I-kappaB kinase/NF-kappaB signaling
BPIBAGO:0043123positive regulation of I-kappaB kinase/NF-kappaB signaling
BPIDAGO:0043123positive regulation of I-kappaB kinase/NF-kappaB signaling
BPTASGO:0043123positive regulation of I-kappaB kinase/NF-kappaB signaling
BPTASGO:0045087innate immune response
BPIDAGO:0045944positive regulation of transcription by RNA polymerase II
BPNASGO:0050852T cell receptor signaling pathway
BPTASGO:0050852T cell receptor signaling pathway
BPIDAGO:0051092positive regulation of NF-kappaB transcription factor activity
BPTASGO:0051092positive regulation of NF-kappaB transcription factor activity
BPTASGO:0051403stress-activated MAPK cascade
BPIMPGO:0051650establishment of vesicle localization
BPTASGO:0070423nucleotide-binding oligomerization domain containing signaling pathway
BPTASGO:0070498interleukin-1-mediated signaling pathway
BPTASGO:1901215negative regulation of neuron death

Gene expression in normal tissue: IKBKG

Gene-model tissue-cancer distribution: Bubble Plot

Gene-drug pathway distribution

Pathways in IKBKG

DatabasePathway IDPathway Des.
pidbcr_5pathwayBCR signaling pathway
pidcd8tcrpathwayTCR signaling in naïve CD8+ T cells
pidfaspathwayFAS (CD95) signaling pathway
pidfcer1pathwayFc-epsilon receptor I signaling in mast cells
pidil1pathwayIL1-mediated signaling events
pidnfkappabcanonicalpathwayCanonical NF-kappaB pathway
pidp75ntrpathwayp75(NTR)-mediated signaling
pidtcr_pathwayTCR signaling in naïve CD4+ T cells
pidtnfpathwayTNF receptor signaling pathway
pidtoll_endogenous_pathwayEndogenous TLR signaling
pidtrail_pathwayTRAIL signaling pathway
kegghsa04010MAPK signaling pathway - Homo sapiens (human)
kegghsa04014Ras signaling pathway - Homo sapiens (human)
kegghsa04062Chemokine signaling pathway - Homo sapiens (human)
kegghsa04064NF-kappa B signaling pathway - Homo sapiens (human)
kegghsa04151PI3K-Akt signaling pathway - Homo sapiens (human)
kegghsa04210Apoptosis - Homo sapiens (human)
kegghsa04380Osteoclast differentiation - Homo sapiens (human)
kegghsa04620Toll-like receptor signaling pathway - Homo sapiens (human)
kegghsa04621NOD-like receptor signaling pathway - Homo sapiens (human)
kegghsa04622RIG-I-like receptor signaling pathway - Homo sapiens (human)
kegghsa04623Cytosolic DNA-sensing pathway - Homo sapiens (human)
kegghsa04625C-type lectin receptor signaling pathway - Homo sapiens (human)
kegghsa04657IL-17 signaling pathway - Homo sapiens (human)
kegghsa04658Th1 and Th2 cell differentiation - Homo sapiens (human)
kegghsa04659Th17 cell differentiation - Homo sapiens (human)
kegghsa04660T cell receptor signaling pathway - Homo sapiens (human)
kegghsa04662B cell receptor signaling pathway - Homo sapiens (human)
kegghsa04668TNF signaling pathway - Homo sapiens (human)
kegghsa04920Adipocytokine signaling pathway - Homo sapiens (human)
kegghsa05120Epithelial cell signaling in Helicobacter pylori infection - Homo sapiens (human)
kegghsa05131Shigellosis - Homo sapiens (human)
kegghsa05142Chagas disease (American trypanosomiasis) - Homo sapiens (human)
kegghsa05145Toxoplasmosis - Homo sapiens (human)
kegghsa05160Hepatitis C - Homo sapiens (human)
kegghsa05161Hepatitis B - Homo sapiens (human)
kegghsa05165Human papillomavirus infection - Homo sapiens (human)
kegghsa05166HTLV-I infection - Homo sapiens (human)
kegghsa05167Kaposi,s sarcoma-associated herpesvirus infection - Homo sapiens (human)
kegghsa05168Herpes simplex infection - Homo sapiens (human)
kegghsa05169Epstein-Barr virus infection - Homo sapiens (human)
kegghsa05200Pathways in cancer - Homo sapiens (human)
kegghsa05203Viral carcinogenesis - Homo sapiens (human)
kegghsa05212Pancreatic cancer - Homo sapiens (human)
kegghsa05215Prostate cancer - Homo sapiens (human)
kegghsa05220Chronic myeloid leukemia - Homo sapiens (human)
kegghsa05221Acute myeloid leukemia - Homo sapiens (human)
kegghsa05222Small cell lung cancer - Homo sapiens (human)
kegghsa05340Primary immunodeficiency - Homo sapiens (human)
kegghsa05418Fluid shear stress and atherosclerosis - Homo sapiens (human)
wikipathwaysWP1433Nucleotide-binding Oligomerization Domain (NOD) pathway
wikipathwaysWP1449Regulation of toll-like receptor signaling pathway
wikipathwaysWP1544MicroRNAs in cardiomyocyte hypertrophy
wikipathwaysWP195IL-1 signaling pathway
wikipathwaysWP2018RANKL-RANK (Receptor activator of NFKB (ligand)) Signaling Pathway
wikipathwaysWP2034Leptin signaling pathway
wikipathwaysWP2112IL17 signaling pathway
wikipathwaysWP23B Cell Receptor Signaling Pathway
wikipathwaysWP231TNF alpha Signaling Pathway
wikipathwaysWP2380Brain-Derived Neurotrophic Factor (BDNF) signaling pathway
wikipathwaysWP262EBV LMP1 signaling
wikipathwaysWP2795Cardiac Hypertrophic Response
wikipathwaysWP288NLR Proteins
wikipathwaysWP3303Rac1-Pak1-p38-MMP-2 pathway
wikipathwaysWP3639Apoptotic Signaling Pathway
wikipathwaysWP382MAPK Signaling Pathway
wikipathwaysWP3849MAPK and NFkB Signalling Pathways Inhibited by Yersinia YopJ
wikipathwaysWP3858Toll-like Receptor Signaling
wikipathwaysWP3863T-Cell antigen Receptor (TCR) pathway during Staphylococcus aureus infection
wikipathwaysWP3865RIG-I-like Receptor Signaling
wikipathwaysWP3878ATM Signaling Network in Development and Disease
wikipathwaysWP3929Chemokine signaling pathway
wikipathwaysWP3932Focal Adhesion-PI3K-Akt-mTOR-signaling pathway
wikipathwaysWP3981miRNA regulation of prostate cancer signaling pathways
wikipathwaysWP4136Fibrin Complement Receptor 3 Signaling Pathway
wikipathwaysWP4172PI3K-Akt Signaling Pathway
wikipathwaysWP4223Ras Signaling
wikipathwaysWP69T-Cell antigen Receptor (TCR) Signaling Pathway
wikipathwaysWP712Estrogen signaling pathway
wikipathwaysWP75Toll-like Receptor Signaling Pathway
reactomeR-HSA-1168372Downstream signaling events of B Cell Receptor (BCR)
reactomeR-HSA-1168372Downstream signaling events of B Cell Receptor (BCR)
reactomeR-HSA-1169091Activation of NF-kappaB in B cells
reactomeR-HSA-1169091Activation of NF-kappaB in B cells
reactomeR-HSA-1236974ER-Phagosome pathway
reactomeR-HSA-1236975Antigen processing-Cross presentation
reactomeR-HSA-1280215Cytokine Signaling in Immune system
reactomeR-HSA-1280218Adaptive Immune System
reactomeR-HSA-1280218Adaptive Immune System
reactomeR-HSA-1606322ZBP1(DAI) mediated induction of type I IFNs
reactomeR-HSA-162582Signal Transduction
reactomeR-HSA-166016Toll Like Receptor 4 (TLR4) Cascade
reactomeR-HSA-166058MyD88:Mal cascade initiated on plasma membrane
reactomeR-HSA-166166MyD88-independent TLR4 cascade
reactomeR-HSA-168138Toll Like Receptor 9 (TLR9) Cascade
reactomeR-HSA-168142Toll Like Receptor 10 (TLR10) Cascade
reactomeR-HSA-168164Toll Like Receptor 3 (TLR3) Cascade
reactomeR-HSA-168176Toll Like Receptor 5 (TLR5) Cascade
reactomeR-HSA-168179Toll Like Receptor TLR1:TLR2 Cascade
reactomeR-HSA-168181Toll Like Receptor 7/8 (TLR7/8) Cascade
reactomeR-HSA-168188Toll Like Receptor TLR6:TLR2 Cascade
reactomeR-HSA-168249Innate Immune System
reactomeR-HSA-168256Immune System
reactomeR-HSA-168256Immune System
reactomeR-HSA-168638NOD1/2 Signaling Pathway
reactomeR-HSA-168643Nucleotide-binding domain, leucine rich repeat containing receptor (NLR) signaling pathways
reactomeR-HSA-168898Toll-Like Receptors Cascades
reactomeR-HSA-168927TICAM1, RIP1-mediated IKK complex recruitment
reactomeR-HSA-168928DDX58/IFIH1-mediated induction of interferon-alpha/beta
reactomeR-HSA-1810476RIP-mediated NFkB activation via ZBP1
reactomeR-HSA-181438Toll Like Receptor 2 (TLR2) Cascade
reactomeR-HSA-1834949Cytosolic sensors of pathogen-associated DNA
reactomeR-HSA-202403TCR signaling
reactomeR-HSA-202424Downstream TCR signaling
reactomeR-HSA-2454202Fc epsilon receptor (FCERI) signaling
reactomeR-HSA-2871837FCERI mediated NF-kB activation
reactomeR-HSA-3108232SUMO E3 ligases SUMOylate target proteins
reactomeR-HSA-392499Metabolism of proteins
reactomeR-HSA-445989TAK1 activates NFkB by phosphorylation and activation of IKKs complex
reactomeR-HSA-446652Interleukin-1 family signaling
reactomeR-HSA-448424Interleukin-17 signaling
reactomeR-HSA-449147Signaling by Interleukins
reactomeR-HSA-450294MAP kinase activation
reactomeR-HSA-450302activated TAK1 mediates p38 MAPK activation
reactomeR-HSA-450321JNK (c-Jun kinases) phosphorylation and activation mediated by activated human TAK1
reactomeR-HSA-4755510SUMOylation of immune response proteins
reactomeR-HSA-5260271Diseases of Immune System
reactomeR-HSA-5357905Regulation of TNFR1 signaling
reactomeR-HSA-5357956TNFR1-induced NFkappaB signaling pathway
reactomeR-HSA-5602358Diseases associated with the TLR signaling cascade
reactomeR-HSA-5602636IKBKB deficiency causes SCID
reactomeR-HSA-5603027IKBKG deficiency causes anhidrotic ectodermal dysplasia with immunodeficiency (EDA-ID) (via TLR)
reactomeR-HSA-5603029IkBA variant leads to EDA-ID
reactomeR-HSA-5607764CLEC7A (Dectin-1) signaling
reactomeR-HSA-5621481C-type lectin receptors (CLRs)
reactomeR-HSA-5684264MAP3K8 (TPL2)-dependent MAPK1/3 activation
reactomeR-HSA-5689880Ub-specific processing proteases
reactomeR-HSA-5689896Ovarian tumor domain proteases
reactomeR-HSA-597592Post-translational protein modification
reactomeR-HSA-73887Death Receptor Signalling
reactomeR-HSA-75893TNF signaling
reactomeR-HSA-9020702Interleukin-1 signaling
reactomeR-HSA-933542TRAF6 mediated NF-kB activation
reactomeR-HSA-933543NF-kB activation through FADD/RIP-1 pathway mediated by caspase-8 and -10
reactomeR-HSA-937039IRAK1 recruits IKK complex
reactomeR-HSA-937041IKK complex recruitment mediated by RIP1
reactomeR-HSA-937061TRIF(TICAM1)-mediated TLR4 signaling
reactomeR-HSA-975138TRAF6 mediated induction of NFkB and MAP kinases upon TLR7/8 or 9 activation
reactomeR-HSA-975144IRAK1 recruits IKK complex upon TLR7/8 or 9 stimulation
reactomeR-HSA-975155MyD88 dependent cascade initiated on endosome
reactomeR-HSA-975871MyD88 cascade initiated on plasma membrane
reactomeR-HSA-983169Class I MHC mediated antigen processing & presentation
reactomeR-HSA-983705Signaling by the B Cell Receptor (BCR)
reactomeR-HSA-983705Signaling by the B Cell Receptor (BCR)
biocartacardiacegfpathwayrole of egf receptor transactivation by gpcrs in cardiac hypertrophy
biocartaceramidepathwayceramide signaling pathway
biocartagsk3pathwayinactivation of gsk3 by akt causes accumulation of b-catenin in alveolar macrophages
biocartail1rpathwaysignal transduction through il1r
biocartakeratinocytepathwaykeratinocyte differentiation
biocartanfkbpathwaynf-kb signaling pathway
biocartanthipathwaynfkb activation by nontypeable hemophilus influenzae
biocartaraccycdpathwayinfluence of ras and rho proteins on g1 to s transition
biocartaranklpathwaybone remodeling
biocartarnapathwaydouble stranded rna induced gene expression
biocartastresspathwaytnf/stress related signaling
biocartatffpathwaytrefoil factors initiate mucosal healing
biocartatidpathwaychaperones modulate interferon signaling pathway
biocartatollpathwaytoll-like receptor pathway

Gene-Drug: Aster Plot

Drug IDDrug NameModel Num.

Gene in drug-gene network: Network Plot

Gene-drug targets distribution

Gene Structure: PDB

Models in IKBKG