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The protein encoded by this gene phosphorylates the inhibitor in the inhibitor/NF-kappa-B complex, causing dissociation of the inhibitor and activation of NF-kappa-B. The encoded protein itself is found in a complex of proteins. Several transcript variants, some protein-coding and some not, have been found for this gene. [provided by RefSeq, Sep 2011]
IKBKB (Inhibitor Of Nuclear Factor Kappa B Kinase Subunit Beta) is a Protein Coding gene. Diseases associated with IKBKB include Immunodeficiency 15A and Immunodeficiency 15B. Among its related pathways are GDNF-Family Ligands and Receptor Interactions and Antigen processing-Cross presentation. Gene Ontology (GO) annotations related to this gene include protein homodimerization activity and protein kinase activity. An important paralog of this gene is CHUK.
IkappaB Kinase (IKK) is an enzyme complex that forms part of the NF-kappaB signaling pathway. The IKK complex is comprised of three subunits (IKKalpha (IKK1), IKKbeta (IKK2) and IKKgamma (NEMO)), the alpha- and beta-subunits are catalytically active whereas the gamma-subunit has a regulatory function.
GO ID | Qualified GO term | Evidence | PubMed IDs |
---|---|---|---|
GO:0000166 | nucleotide binding | IEA | -- |
GO:0004672 | protein kinase activity | IEA,IDA | 20434986 |
GO:0004674 | protein serine/threonine kinase activity | TAS | -- |
GO:0005515 | protein binding | IEA,IPI | 9520446 |
GO:0005524 | ATP binding | IEA | -- |
GO ID | Qualified GO term | Evidence | PubMed IDs |
---|---|---|---|
GO:0005634 | nucleus | IEA | -- |
GO:0005737 | cytoplasm | IBA | 21873635 |
GO:0005829 | cytosol | IDA,TAS | -- |
GO:0008385 | IkappaB kinase complex | IBA,TAS | 18626576 |
GO:0009898 | cytoplasmic side of plasma membrane | ISS | -- |
SuperPathway | Contained pathways | ||
---|---|---|---|
1 | Activated TLR4 signalling | ||
2 | TNFR1 Pathway |
DR3 Signaling
.87
TWEAK Pathway
.87
TNFR1 Pathway
.56
TNF Signaling
.55
Apoptosis and survival TNFR1 signaling pathway
.52
GITR Pathway
.46
Apoptotic Pathways Triggered By HIV1
.42
|
Apoptosis through Death Receptors
.42
TRAIL Pathway
.40
Fas Signaling
.35
|
3 | 4-1BB Pathway |
4-1BB Pathway
.54
CD40 Signaling
.54
CD27 Pathway
.48
CMV and MAPK Pathways
.46
NF-KappaB Activation by EBV
.42
|
NF-kappaB Activation by Viruses
.42
TNFR2 Pathway
.37
PKR Pathway
.36
SUMO Pathway
.30
|
4 | Cytosolic sensors of pathogen-associated DNA | ||
5 | Toll-like Receptor Signaling Pathway |
.37
.34
|
GO ID | Qualified GO term | Evidence | PubMed IDs |
---|---|---|---|
GO:0002223 | stimulatory C-type lectin receptor signaling pathway | TAS | -- |
GO:0002479 | antigen processing and presentation of exogenous peptide antigen via MHC class I, TAP-dependent | TAS | -- |
GO:0002756 | MyD88-independent toll-like receptor signaling pathway | TAS | -- |
GO:0006468 | protein phosphorylation | IEA,NAS | 9346484 |
GO:0006954 | inflammatory response | TAS | 18626576 |
Name | Status | Disease Links | Group | Role | Mechanism of Action | Clinical Trials |
---|---|---|---|---|---|---|
Arsenic trioxide | Approved, Investigational | Pharma | Target, inducer, inhibitor | 131 | ||
Auranofin | Approved, Investigational | Pharma | Activator, Activation, Target, inhibitor | Thioredoxin reductase inhibitor; induces MPT | 12 | |
Sulfasalazine | Approved | Pharma | Inhibitor, Inhibition, Target, inhibitor | NF-κB activation inhibitor | 84 | |
Mesalazine | Approved | Pharma | Target, inhibitor | 0 | ||
Aspirin | Approved, Vet_approved | Pharma | Channel blocker, Target | 1512 |
Name | Synonyms | Role | CAS Number | PubChem IDs | PubMed IDs | |
---|---|---|---|---|---|---|
ADP |
|
Agonist, Full agonist, Partial agonist, Gating inhibitor, Antagonist | 58-64-0 | |||
IKK 16 |
|
1186195-62-9 |
|
|
Compound | Action | Cas Number |
---|---|---|
ACHP | IκB kinase inhibitor | 406208-42-2 |
Andrographolide | NF-κB signaling Inhibitor | 5508-58-7 |
Bardoxolone methyl | IKK inhibitor, potent antioxidant inflammation modulator | 218600-53-4 |
Bay 11-7085 | NK-κB activation inhibitor | 196309-76-9 |
Bay 11-7821(BAY 11-7082) | IKK/NF-κB/TNFα inhibitor | 19542-67-7 |
Bay 65-1942 HCl salt | 600734-06-3 | |
BMS-345541 | IKK inhibitor,highly selective | 547757-23-3 |
BMS-345541(free base) | IKK-1/IKK-2 inhibitor,potent and selective | 445430-58-0 |
Choline Fenofibrate | Choline salt of fenofibric acid | 856676-23-8 |
Honokiol | Antioxidant/antitumor/antiangiogenic agent | 35354-74-6 |
IKK-16 (IKK Inhibitor VII) | Selective IκB kinase inhibitor | 873225-46-8 |
IKK-2 inhibitor VIII | IKK-2 inhibitor,potent and selective | 406209-26-5 |
IMD 0354 | 978-62-1 | |
Luteolin | Antioxidant and free radical scavenger | 491-70-3 |
LY2409881 | 946518-60-1 | |
ML 120B dihydrochloride | ||
MLN120B | IκB Kinase β Inhibitor | 783348-36-7 |
PF 184 | 1187460-81-6 | |
PS 1145 dihydrochloride | 1049743-58-9 | |
SC-514 | ATP-competitive IKK-2 inhibitor, orally active | 354812-17-2 |
Tanshinone IIA | Antioxidant and anti-inflammatory reagent | 568-72-9 |
TPCA-1 | IKK-2 inhibitor,potent and selective | 507475-17-4 |
WS6 | 1421227-53-3 |
ExUns: | 1a | · | 1b | · | 1c | ^ | 2a | · | 2b | ^ | 3 | ^ | 4 | ^ | 5 | ^ | 6 | ^ | 7 | ^ | 8 | ^ | 9 | ^ | 10 | ^ | 11 | ^ | 12a | · | 12b | ^ | 13 | ^ | 14 | ^ | 15 | ^ | 16 | ^ | 17a | · | 17b | · | 17c | ^ | 18 | ^ | 19 | ^ | 20a | · |
---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
SP1: | - | - | - | - | - | |||||||||||||||||||||||||||||||||||||||||||||||
SP2: | - | - | - | - | - | |||||||||||||||||||||||||||||||||||||||||||||||
SP3: | - | - | - | - | - | - | - | - | - | - | - | |||||||||||||||||||||||||||||||||||||||||
SP4: | - | - | ||||||||||||||||||||||||||||||||||||||||||||||||||
SP5: | - | |||||||||||||||||||||||||||||||||||||||||||||||||||
SP6: | ||||||||||||||||||||||||||||||||||||||||||||||||||||
SP7: | ||||||||||||||||||||||||||||||||||||||||||||||||||||
SP8: |
ExUns: | 20b | ^ | 21 | ^ | 22a | · | 22b | ^ | 23a | · | 23b | · | 23c |
---|---|---|---|---|---|---|---|---|---|---|---|---|---|
SP1: | - | ||||||||||||
SP2: | - | ||||||||||||
SP3: | - | ||||||||||||
SP4: | |||||||||||||
SP5: | |||||||||||||
SP6: | - | ||||||||||||
SP7: | - | ||||||||||||
SP8: |
This gene was present in the common ancestor of animals.
Organism | Taxonomy | Gene | Similarity | Type | Details |
---|---|---|---|---|---|
Chimpanzee (Pan troglodytes) |
Mammalia | IKBKB 30 31 |
|
OneToOne | |
Dog (Canis familiaris) |
Mammalia | IKBKB 30 31 |
|
OneToOne | |
Platypus (Ornithorhynchus anatinus) |
Mammalia | IKBKB 31 |
|
OneToOne | |
Oppossum (Monodelphis domestica) |
Mammalia | IKBKB 31 |
|
OneToOne | |
Mouse (Mus musculus) |
Mammalia | Ikbkb 30 17 31 |
|
OneToOne | |
Rat (Rattus norvegicus) |
Mammalia | Ikbkb 30 |
|
||
Cow (Bos Taurus) |
Mammalia | IKBKB 30 31 |
|
OneToOne | |
Chicken (Gallus gallus) |
Aves | IKBKB 30 31 |
|
OneToOne | |
Lizard (Anolis carolinensis) |
Reptilia | IKBKB 31 |
|
OneToOne | |
Tropical Clawed Frog (Silurana tropicalis) |
Amphibia | ikbkb 30 |
|
||
Str.17591 30 |
|
||||
Zebrafish (Danio rerio) |
Actinopterygii | ikbkb 30 31 |
|
OneToOne | |
Fruit Fly (Drosophila melanogaster) |
Insecta | ird5 31 32 |
|
OneToMany |
SNP ID | Clinical significance and condition | Chr 08 pos | Variation | AA Info | Type |
---|---|---|---|---|---|
638981 | Uncertain Significance: Immunodeficiency 15 | 42,320,832(+) | C/T | MISSENSE_VARIANT,NON_CODING_TRANSCRIPT_VARIANT | |
641287 | Uncertain Significance: Immunodeficiency 15 | 42,290,279(+) | G/T | INTRON_VARIANT | |
641937 | Uncertain Significance: Immunodeficiency 15 | 42,326,062(+) | G/A | NON_CODING_TRANSCRIPT_VARIANT,SYNONYMOUS_VARIANT | |
646483 | Uncertain Significance: Immunodeficiency 15 | 42,329,128(+) | G/A | MISSENSE_VARIANT,NON_CODING_TRANSCRIPT_VARIANT | |
649552 | Uncertain Significance: Immunodeficiency 15 | 42,318,648(+) | G/A | MISSENSE_VARIANT,NON_CODING_TRANSCRIPT_VARIANT |
Variant ID | Type | Subtype | PubMed ID |
---|---|---|---|
dgv128n16 | CNV | deletion | 17901297 |
dgv36e209 | CNV | deletion | 24192839 |
dgv7185n100 | CNV | gain | 25217958 |
esv1003654 | CNV | deletion | 20482838 |
esv1761681 | CNV | deletion | 17803354 |
esv2736935 | CNV | deletion | 23290073 |
esv3543424 | CNV | deletion | 23714750 |
esv3543425 | CNV | deletion | 23714750 |
esv3617007 | CNV | loss | 21293372 |
nsv1132145 | CNV | deletion | 24896259 |
nsv1145767 | CNV | deletion | 26484159 |
nsv398024 | CNV | deletion | 16902084 |
nsv476099 | CNV | novel sequence insertion | 20440878 |
nsv508508 | CNV | deletion | 20534489 |
nsv611131 | CNV | gain | 21841781 |
nsv6169 | CNV | deletion | 18451855 |
nsv831301 | CNV | loss | 17160897 |
Disorder | Aliases | PubMed IDs |
---|---|---|
immunodeficiency 15a |
|
|
immunodeficiency 15b |
|
|
severe combined immunodeficiency due to ikk2 deficiency |
|
|
incontinentia pigmenti |
|
|
hepatitis |
|
|