Seroatlas · Human Serome Atlas

IKBKG

NF-kappa-B essential modulator

Also known as: FIP-3, FIP3, Fip3p, IKK-gamma, IKKAP1, IKKG, IP1, IP2, NEMO, NEMO_HUMAN, ZC2HC9

Cross-references: UniProt · Ensembl · Human Protein Atlas · GeneCards · NCBI Gene

Protein identityUniProt · HPA

UniProt accession
Q9Y6K9
Gene
IKBKG
Ensembl
ENSG00000269335
Chromosome
X
Canonical length
419 aa
Protein class
Disease related genes, Human disease related genes, Predicted intracellular proteins, RAS pathway related proteins
Subcellular location
Cytosol
Quaternary structure
Homodimer

OverviewNCBI Gene

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]

Canonical amino-acid sequenceUniProt

419 residues, UniProt reviewed canonical sequence.

>Q9Y6K9|IKBKG
     1  MNRHLWKSQL CEMVQPSGGP AADQDVLGEE SPLGKPAMLH LPSEQGAPET LQRCLEENQE
    61  LRDAIRQSNQ ILRERCEELL HFQASQREEK EFLMCKFQEA RKLVERLGLE KLDLKRQKEQ
   121  ALREVEHLKR CQQQMAEDKA SVKAQVTSLL GELQESQSRL EAATKECQAL EGRARAASEQ
   181  ARQLESEREA LQQQHSVQVD QLRMQGQSVE AALRMERQAA SEEKRKLAQL QVAYHQLFQE
   241  YDNHIKSSVV GSERKRGMQL EDLKQQLQQA EEALVAKQEV IDKLKEEAEQ HKIVMETVPV
   301  LKAQADIYKA DFQAERQARE KLAEKKELLQ EQLEQLQREY SKLKASCQES ARIEDMRKRH
   361  VEVSQAPLPP APAYLSSPLA LPSQRRSPPE EPPDFCCPKC QYQAPDMDTL QIHVMECIE

LocalizationUniProt · AlphaFold · HPA

Whether an antibody against IKBKG can act on the native protein depends on physical access: surface and secreted proteins are reachable by circulating antibodies, intracellular proteins usually are not.

Antibody reachability
Intracellular
Secreted
No
Transmembrane segments
0
Mean surface accessibility (rSASA)
0.53
Highest tissue expression
63 nTPM

Expression across tissuesHPA

Tissue

  • liver: 63 nTPM
  • spleen: 43 nTPM
  • adrenal gland: 29 nTPM
  • bone marrow: 27 nTPM
  • lung: 27 nTPM
  • esophagus: 25 nTPM

Single-cell type

  • tuft cells: 23 nCPM
  • platelets: 16 nCPM
  • neutrophils: 11 nCPM
  • retinal horizontal cells: 10 nCPM
  • rod photoreceptor cells: 9.7 nCPM
  • distal convoluted tubule cells: 8.4 nCPM

Immune cell

  • eosinophil: 60 nTPM
  • neutrophil: 49 nTPM
  • non-classical monocyte: 48 nTPM
  • intermediate monocyte: 40 nTPM
  • gdT-cell: 34 nTPM
  • classical monocyte: 31 nTPM

Brain region

  • medulla oblongata: 32 nTPM
  • white matter: 30 nTPM
  • pons: 29 nTPM
  • cerebral cortex: 29 nTPM
  • thalamus: 28 nTPM
  • midbrain: 25 nTPM

DiseaseUniProt · ClinVar · IEDB · PubMed

Four sources answering four different questions about IKBKG.

Disease | AllUniProt

Conditions IKBKG is implicated in, by any mechanism.

  • Ectodermal dysplasia and immunodeficiency 1 (EDAID1) MIM:300291
  • Immunodeficiency 33 (IMD33) MIM:300636
  • Incontinentia pigmenti (IP) MIM:308300
  • Autoinflammatory disease, systemic, X-linked (SAIDX) MIM:301081

Disease | GeneticClinVar

75 pathogenic / likely-pathogenic of 174 ClinVar records.

Conditions with pathogenic or likely-pathogenic variants.

  • Incontinentia pigmenti syndrome
  • Ectodermal dysplasia and immunodeficiency 1
  • Immunodeficiency 33
  • ECTODERMAL DYSPLASIA AND IMMUNODEFICIENCY 1, MALE-RESTRICTED
  • Autoinflammatory disease, X-linked

Genetic constraint and essentialitygnomAD · DepMap

Does the body need this protein intact? Low LOEUF or a strong DepMap dependency means loss or blockade of the protein is likely to be felt.

gnomAD LOEUF (loss-of-function intolerance)
1.37
gnomAD pLI
0.08
gnomAD missense Z
0.03
DepMap mean gene effect
-0.15
DepMap dependency class
selective

Cancer expressionTCGA

Across TCGA tumor cohorts, this protein is over-expressed in roughly 7% of surveyed tumor types (aggregate summary; per-cohort expression, alteration, and survival load in the interactive view).

OntologyGO

Biological processes

Molecular functions

Cellular components

Protein domainsUniProt · Pfam · InterPro

KeywordsUniProt

InteractionsUniProt · HPA

Protein binding partners of IKBKG in the human serome: UniProt-annotated complex subunits plus reported interactors. Each links to its own Seroatlas record.

Antibody and autoantibody relevanceSeroatlas analysis

Seroatlas reads IKBKG as an antibody target. Whether an autoantibody or antibody against IKBKG could matter depends on whether native IKBKG is physically reachable, whether the body needs it intact, and whether it acts in a disease-relevant tissue.

IKBKG is annotated as predominantly intracellular. Intracellular proteins are common autoantibody markers, becoming visible to the immune system after cell injury or altered processing, but are usually markers of disease rather than direct drivers.

Annotation status

The present source text does not explicitly label IKBKG as an autoantigen. Seroatlas presents hypothesis context only and does not manufacture a known-serology claim.

Canonical record: https://seroatlas.com/gene/IKBKG. Study-independent annotations aggregated from UniProt, Human Protein Atlas, PubMed, IEDB, Pfam, InterPro, Gene Ontology, AlphaFold, gnomAD, DepMap, ClinVar, TCGA. Catalog release seroatlas-reviewed-human-uniprot-20260313.

Seroatlas is the reference for exploring autoantibody and antibody serology at the human-protein level: the autoreactome and human serome, multiplex serology (HuProt, HuScan, VirScan, PhIP-Seq).

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