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10 sources retrieved · Most recent: April 2026 · Index updated 14 days ago
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NFKBIB
NFKB inhibitor beta
Chromosome 19 · 19q13.2
NCBI Gene: 4793Ensembl: ENSG00000104825.17HGNC: HGNC:7798UniProt: G5E9C2
102PubMed Papers
20Diseases
0Drugs
0Pathogenic Variants
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
protein bindingcellular response to lipopolysaccharidesignal transductiontranscription coactivator activitydengue diseasedementiagastrointestinal stromal tumorisolated agammaglobulinemia
✦AI Summary

NFKBIB (NF-κB inhibitor beta) functions as a regulatory protein that controls NF-κB transcription factor activity through cytoplasmic sequestration 1. In unstimulated cells, NFKBIB binds NF-κB and masks its nuclear localization signal, preventing nuclear translocation 1. Upon cell stimulation, NFKBIB undergoes phosphorylation and degradation, releasing NF-κB to translocate to the nucleus where it activates transcription of immune and inflammatory response genes 1. Notably, newly synthesized unphosphorylated NFKBIB can rebind NF-κB and protect it from further inactivation, allowing nuclear NF-κB function 1. Association with NKIRAS proteins increases NFKBIB resistance to degradation, prolonging its inhibitory effects. NFKBIB dysfunction contributes to multiple disease pathologies. Dysregulation is implicated in inflammatory conditions, where elevated NF-κB signaling drives myeloid bias and inflammatory responses 2. In gastrointestinal stromal tumors, nuclear KIT drives NFKBIB expression, creating an autoregulatory loop that maintains KIT expression and imatinib resistance 3. In gastric cancer, miR-20a-mediated NFKBIB downregulation enhances NF-κB activation and promotes chemoresistance through upregulation of anti-apoptotic factors 4. NFKBIB upregulation has been identified as a hub gene in dilated cardiomyopathy 5 and in cytokine storm-related inflammatory diseases 6, suggesting its role in pathological inflammation.

Sources cited
1
NFKBIB binds NF-κB, masks its nuclear localization signal, and prevents nuclear uptake; undergoes phosphorylation and degradation upon cell stimulation; newly synthesized NFKBIB can rebind and protect NF-κB
PMID: 10837071
2
NFKBIB deletion in IκB- mice model demonstrates role in chronic inflammation and myeloid bias through elevated NF-κB activity
PMID: 39791596
3
Nuclear KIT binds NFKBIB promoter and enhances its expression; NFKBIB downregulation leads to RELA nuclear translocation and reduced KIT expression in imatinib-resistant GISTs
PMID: 31363162
4
miR-20a targets NFKBIB to promote NF-κB activation and chemoresistance in gastric cancer through increased anti-apoptotic factor expression
PMID: 26286834
5
NFKBIB identified as hub gene upregulated in dilated cardiomyopathy in both bioinformatic analysis and experimental validation
PMID: 40074718
6
NFKBIB significantly upregulated in iMCD-TAFRO/NOS subtype associated with cytokine storm and enhanced inflammatory signaling
PMID: 39462545
Disease Associationsⓘ20
dengue diseaseOpen Targets
0.37Weak
dementiaOpen Targets
0.16Weak
gastrointestinal stromal tumorOpen Targets
0.07Suggestive
isolated agammaglobulinemiaOpen Targets
0.05Suggestive
SepsisOpen Targets
0.05Suggestive
immunodeficiency 18Open Targets
0.04Suggestive
common variable immunodeficiencyOpen Targets
0.04Suggestive
immunodeficiency 112Open Targets
0.04Suggestive
autoimmune diseaseOpen Targets
0.04Suggestive
combined immunodeficiency with skin granulomasOpen Targets
0.04Suggestive
polyarticular juvenile idiopathic arthritis, rheumatoid factor positiveOpen Targets
0.04Suggestive
immunodeficiency, common variable, 3Open Targets
0.04Suggestive
agammaglobulinemia 8, autosomal dominantOpen Targets
0.04Suggestive
immunodeficiency 99 with hypogammaglobulinemia and autoimmune cytopeniasOpen Targets
0.04Suggestive
activated PI3K-delta syndromeOpen Targets
0.04Suggestive
immunodeficiency 62Open Targets
0.04Suggestive
hyper-IgE recurrent infection syndrome 5, autosomal recessiveOpen Targets
0.04Suggestive
immunodeficiency 73c with defective neutrophil chemotaxis and hypogammaglobulinemiaOpen Targets
0.04Suggestive
immunodeficiency, common variable, 4Open Targets
0.04Suggestive
BENTA diseaseOpen Targets
0.04Suggestive
Pathogenic Variants
No pathogenic variants reported on ClinVar for this gene.
View on ClinVar ↗
Related Genes
CHUKProtein interaction100%IKBKBProtein interaction100%NFKB1Protein interaction100%NFKB2Protein interaction100%UBE2D2Protein interaction100%SKP1Protein interaction100%
Tissue Expression6 tissues
Liver
100%
Bone Marrow
70%
Lung
68%
Brain
37%
Ovary
35%
Heart
31%
Gene Interaction Network
Click a node to explore
NFKBIBCHUKIKBKBNFKB1NFKB2UBE2D2SKP1
PROTEIN STRUCTURE
Preparing viewer…
PDB9CK0 · 2.60 Å · X-ray
View on RCSB ↗
Constraintⓘ
LOEUFⓘ
0.83LoF Tolerant
pLIⓘ
0.00Tolerant
Observed/Expected LoF0.55 [0.37–0.83]
RankingsWhere NFKBIB stands among ~20K protein-coding genes
  • #4,682of 20,598
    Most Researched102 · top quartile
  • #7,095of 17,882
    Most Constrained (LOEUF)0.83
Genes detectedNFKBIB
Sources retrieved10 papers
Response time—
📄 Sources
10▼
1
Shared principles in NF-kappaB signaling.
PMID: 18267068
Cell · 2008
1.00
2
NF-kappaB regulation in the immune system.
PMID: 12360211
Nat Rev Immunol · 2002
0.90
3
Phosphorylation meets ubiquitination: the control of NF-[kappa]B activity.
PMID: 10837071
Annu Rev Immunol · 2000
0.80
4
Population dynamics modeling reveals that myeloid bias involves both HSC differentiation and progenitor proliferation biases.
PMID: 39791596
Blood · 2025
0.70
5
Discovering new hub genes of dilated cardiomyopathy.
PMID: 40074718
ESC Heart Fail · 2025
0.60