Version 5.4
Homo sapiens Gene: IKBKG
Summary
InnateDB Gene IDBG-777831.1
Last Modified 2014-10-13 [Report errors or provide feedback]
Gene Symbol IKBKG
Gene Name inhibitor of kappa light polypeptide gene enhancer in B-cells, kinase gamma
Synonyms AMCBX1; FIP-3; FIP3; Fip3p; IKK-gamma; IMD33; IP; IP1; IP2; IPD2; NEMO; ZC2HC9
Species Homo sapiens
Ensembl Gene ENSG00000269335
Encoded Proteins
IDBP-813541
inhibitor of kappa light polypeptide gene enhancer in B-cells, kinase gamma
IDBP-813542
inhibitor of kappa light polypeptide gene enhancer in B-cells, kinase gamma
IDBP-813543
inhibitor of kappa light polypeptide gene enhancer in B-cells, kinase gamma
IDBP-813546
inhibitor of kappa light polypeptide gene enhancer in B-cells, kinase gamma
IDBP-813547
inhibitor of kappa light polypeptide gene enhancer in B-cells, kinase gamma
IDBP-813548
inhibitor of kappa light polypeptide gene enhancer in B-cells, kinase gamma
IDBP-813549
inhibitor of kappa light polypeptide gene enhancer in B-cells, kinase gamma
IDBP-813551
inhibitor of kappa light polypeptide gene enhancer in B-cells, kinase gamma
IDBP-813552
inhibitor of kappa light polypeptide gene enhancer in B-cells, kinase gamma
Protein Structure
Useful resources Stemformatics EHFPI ImmGen
InnateDB Annotation
Summary
PMID 10968790
IKBKG is the regulatory subunit of the IKK-signalosome complex that is required for proinflammatory activation of the I-kappaB-kinase (IKK) complex.
PMID 19136968
IKBKG is linearly polyubiquitinated by the LUBAC ligase complex, consisting of RNF31 and RBCK1 proteins, to regulate the canonical NF-kappaB activation pathway.
PMID 18080803
IKBKG regulates TNF alpha signaling by coordinating cell responses mediated by the AP-1 and NF-kappa B pathways.
PMID 20010814
IKBKG is essential for NF-kappaB activation and the polyubiquitylation and the degradation of IKBKG during Shigella infection is a new bacterial strategy to modulate host inflammatory responses.
PMID 23951545
The binding of MAVS to Traf2, Traf5, and Traf6 is dependent on virus infection and MAVS polymerization. The TRAF proteins promote ubiquitination that recruits IKBKG binding to the MAVS signalling complex
Entrez Gene
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. 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. [provided by RefSeq, Aug 2011]
Gene Information
Type Protein coding
Genomic Location Chromosome X:154541199-154565046
Strand Forward strand
Band q28
Transcripts
ENST00000618670 ENSP00000483825
ENST00000612051 ENSP00000480431
ENST00000594239 ENSP00000471166
ENST00000619941 ENSP00000478979
ENST00000615874 ENSP00000483381
ENST00000617207 ENSP00000484023
ENST00000611176 ENSP00000478616
ENST00000615186 ENSP00000479144
ENST00000611071 ENSP00000479662
ENST00000617838
Interactions
Number of Interactions This gene and/or its encoded proteins are associated with 545 experimentally validated interaction(s) in this database.
Experimentally validated
Total 545 [view]
Protein-Protein 543 [view]
Protein-DNA 2 [view]
Protein-RNA 0
DNA-DNA 0
RNA-RNA 0
DNA-RNA 0
Gene Ontology

Molecular Function
Accession GO Term
GO:0004871 signal transducer activity
GO:0005515 protein binding
GO:0019904 protein domain specific binding
GO:0042803 protein homodimerization activity
GO:0042975 peroxisome proliferator activated receptor binding
GO:0046872 metal ion binding
GO:0046982 protein heterodimerization activity
Biological Process
GO:0000187 activation of MAPK activity
GO:0001782 B cell homeostasis
GO:0002224 toll-like receptor signaling pathway
GO:0002755 MyD88-dependent toll-like receptor signaling pathway
GO:0002756 MyD88-independent toll-like receptor signaling pathway
GO:0006351 transcription, DNA-templated
GO:0006915 apoptotic process
GO:0006954 inflammatory response
GO:0006955 immune response
GO:0006974 cellular response to DNA damage stimulus
GO:0007249 I-kappaB kinase/NF-kappaB signaling
GO:0007250 activation of NF-kappaB-inducing kinase activity
GO:0007254 JNK cascade
GO:0009615 response to virus
GO:0016032 viral process
GO:0032481 positive regulation of type I interferon production
GO:0034134 toll-like receptor 2 signaling pathway
GO:0034138 toll-like receptor 3 signaling pathway
GO:0034142 toll-like receptor 4 signaling pathway
GO:0034146 toll-like receptor 5 signaling pathway
GO:0034162 toll-like receptor 9 signaling pathway
GO:0034166 toll-like receptor 10 signaling pathway
GO:0035666 TRIF-dependent toll-like receptor signaling pathway
GO:0035872 nucleotide-binding domain, leucine rich repeat containing receptor signaling pathway
GO:0038095 Fc-epsilon receptor signaling pathway
GO:0038123 toll-like receptor TLR1:TLR2 signaling pathway
GO:0038124 toll-like receptor TLR6:TLR2 signaling pathway
GO:0043123 positive regulation of I-kappaB kinase/NF-kappaB signaling
GO:0045087 innate immune response (InnateDB)
GO:0045944 positive regulation of transcription from RNA polymerase II promoter
GO:0050852 T cell receptor signaling pathway
GO:0051092 positive regulation of NF-kappaB transcription factor activity
GO:0051403 stress-activated MAPK cascade
GO:0070423 nucleotide-binding oligomerization domain containing signaling pathway
Cellular Component
GO:0005622 intracellular
GO:0005634 nucleus
GO:0005737 cytoplasm
GO:0005829 cytosol
GO:0008385 IkappaB kinase complex
Orthologs
No orthologs found for this gene
Pathways
NETPATH
NetPath_9
TNFalpha pathway
NetPath_12
BCR pathway
NetPath_13
IL1 pathway
NetPath_21
RANKL pathway
NetPath_22
Leptin pathway
REACTOME
REACT_118563
RIP-mediated NFkB activation via ZBP1 pathway
REACT_118764
ZBP1(DAI) mediated induction of type I IFNs pathway
REACT_24969
TRAF6 mediated NF-kB activation pathway
REACT_25039
NF-kB activation through FADD/RIP-1 pathway mediated by caspase-8 and -10 pathway
REACT_25359
RIG-I/MDA5 mediated induction of IFN-alpha/beta pathways pathway
REACT_21368
JNK (c-Jun kinases) phosphorylation and activation mediated by activated human TAK1 pathway
REACT_21399
activated TAK1 mediates p38 MAPK activation pathway
REACT_25374
IKK complex recruitment mediated by RIP1 pathway
REACT_21281
TAK1 activates NFkB by phosphorylation and activation of IKKs complex pathway
REACT_6809
MyD88-independent cascade pathway
REACT_6783
Toll Like Receptor 3 (TLR3) Cascade pathway
REACT_24918
IRAK1 recruits IKK complex pathway
REACT_6788
MyD88:Mal cascade initiated on plasma membrane pathway
REACT_8005
Toll Like Receptor TLR1:TLR2 Cascade pathway
REACT_8006
Toll Like Receptor TLR6:TLR2 Cascade pathway
REACT_25354
IRAK1 recruits IKK complex upon TLR7/8 or 9 stimulation pathway
REACT_25024
TRAF6 mediated induction of NFkB and MAP kinases upon TLR7/8 or 9 activation pathway
REACT_25222
MyD88 dependent cascade initiated on endosome pathway
REACT_9047
Toll Like Receptor 9 (TLR9) Cascade pathway
REACT_27215
MyD88 cascade initiated on plasma membrane pathway
REACT_9027
Toll Like Receptor 10 (TLR10) Cascade pathway
REACT_6894
Toll Like Receptor 4 (TLR4) Cascade pathway
REACT_9061
Toll Like Receptor 5 (TLR5) Cascade pathway
REACT_75776
NOD1/2 Signaling Pathway pathway
REACT_12555
Downstream TCR signaling pathway
REACT_22442
Interleukin-1 signaling pathway
REACT_118656
Activation of NF-kappaB in B cells pathway
REACT_163994
FCERI mediated NF-kB activation pathway
REACT_9020
Toll Like Receptor 7/8 (TLR7/8) Cascade pathway
REACT_12526
TCR signaling pathway
REACT_75790
Cytokine Signaling in Immune system pathway
REACT_6802
Innate Immune System pathway
REACT_7980
Toll Like Receptor 2 (TLR2) Cascade pathway
REACT_6966
Toll-Like Receptors Cascades pathway
REACT_163936
Fc epsilon receptor (FCERI) signaling pathway
REACT_21308
MAP kinase activation in TLR cascade pathway
REACT_75913
Nucleotide-binding domain, leucine rich repeat containing receptor (NLR) signaling pathways pathway
REACT_75774
Adaptive Immune System pathway
REACT_6900
Immune System pathway
REACT_22232
Signaling by Interleukins pathway
REACT_6890
Activated TLR4 signalling pathway
REACT_25281
TRIF-mediated TLR3/TLR4 signaling pathway
REACT_118823
Cytosolic sensors of pathogen-associated DNA pathway
REACT_118773
Signaling by the B Cell Receptor (BCR) pathway
REACT_118638
Downstream signaling events of B Cell Receptor (BCR) pathway
KEGG
hsa05221
Acute myeloid leukemia pathway
hsa05120
Epithelial cell signaling in Helicobacter pylori infection pathway
hsa04210
Apoptosis pathway
hsa04010
MAPK signaling pathway pathway
hsa05222
Small cell lung cancer pathway
hsa04662
B cell receptor signaling pathway pathway
hsa05215
Prostate cancer pathway
hsa04660
T cell receptor signaling pathway pathway
hsa04620
Toll-like receptor signaling pathway pathway
hsa05220
Chronic myeloid leukemia pathway
hsa04920
Adipocytokine signaling pathway pathway
hsa05212
Pancreatic cancer pathway
hsa05340
Primary immunodeficiency pathway
hsa04062
Chemokine signaling pathway pathway
hsa05200
Pathways in cancer pathway
hsa04622
RIG-I-like receptor signaling pathway pathway
hsa04621
NOD-like receptor signaling pathway pathway
hsa04623
Cytosolic DNA-sensing pathway pathway
hsa05142
Chagas disease (American trypanosomiasis) pathway
hsa04380
Osteoclast differentiation pathway
hsa05131
Shigellosis pathway
hsa05145
Toxoplasmosis pathway
hsa05160
Hepatitis C pathway
INOH
PID NCI
cd8tcrpathway
TCR signaling in naïve CD8+ T cells
tcr_pathway
TCR signaling in naïve CD4+ T cells
il1pathway
IL1-mediated signaling events
tnfpathway
TNF receptor signaling pathway
trail_pathway
TRAIL signaling pathway
p75ntrpathway
p75(NTR)-mediated signaling
bcr_5pathway
BCR signaling pathway
nfkappabcanonicalpathway
Canonical NF-kappaB pathway
fcer1pathway
Fc-epsilon receptor I signaling in mast cells
toll_endogenous_pathway
Endogenous TLR signaling
faspathway
FAS (CD95) signaling pathway
Cross-References
SwissProt
TrEMBL C9J1R1 D3DWY0
UniProt Splice Variant
Entrez Gene 8517
UniGene
RefSeq NM_001099856 NM_001099857 NM_001145255 NM_003639 XM_005274760 XM_005274763 XM_005274764 XM_006724853
HUGO HGNC:5961
OMIM 300248
CCDS CCDS14757 CCDS48196 CCDS48197
HPRD
IMGT
EMBL AF277315 CH471172
GenPept EAW72679 EAW72680 EAW72681
RNA Seq Atlas 8517

If you use InnateDB for your research, please cite the following publication:

Breuer et al., InnateDB: systems biology of innate immunity and beyond - recent updates and continuing curation. Nucl. Acids Res. (2013) 41 (D1) 

InnateDB is being developed jointly by the Brinkman Laboratory (Simon Fraser University, British Columbia, Canada), the Hancock Laboratory (University of British Columbia, Vancouver, British Columbia) and the Lynn EMBL Australia Group (South Australian Health & Medical Research Institute and Flinders University, Adelaide, Australia).

Funding is currently provided by Allergen and EMBL Australia. Previous funding has been provided by Genome Canada, the Foundation for the National Institutes of Health through the Grand Challenges in Global Health initiative and by Teagasc. InnateDB curated interactions are licensed under the Design Science License. All other data is licensed under the terms of the originating database. Contact: innatedb-mail@sfu.ca