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GeneE
10 sources retrieved · Most recent: April 2026 · Index updated 15 days ago
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GBP3
guanylate binding protein 3
Chromosome 1 · 1p22.2
NCBI Gene: 2635Ensembl: ENSG00000117226.13HGNC: HGNC:4184UniProt: A0ABB0MVI2
23PubMed Papers
20Diseases
0Drugs
0Pathogenic Variants
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
protein bindingcellular response to interleukin-1cellular response to tumor necrosis factoridentical protein bindingbacterial diseaseisolated agammaglobulinemiaImmunodeficiency due to a late component of complements deficiencyrecurrent infections associated with rare immunoglobulin isotypes deficiency
✦AI Summary

GBP3 is an interferon-γ-inducible GTPase that functions as a critical component of intracellular innate immunity, with context-dependent roles in both antimicrobial defense and pathological inflammation. In antimicrobial responses, GBP3 governs caspase-4 activation on cytosolic bacterial surfaces by assembling polyvalent signaling platforms with other GBPs, ultimately triggering gasdermin-D-dependent pyroptosis and interleukin-18 processing to eliminate intracellular bacteria 1. GBP3 directly binds lipopolysaccharide of Gram-negative bacteria and localizes to intracellular microbes through membrane binding and protein trafficking 2. Beyond antimicrobial immunity, GBP3 participates in cancer-associated pathways. In glioblastoma, GBP3 promotes cell proliferation via the SQSTM1/p62-ERK1/2 axis 3 and enhances chemotherapy resistance by stabilizing STING protein, which upregulates DNA damage repair pathways including NRF2 and MGMT expression 4. Pan-cancer analyses reveal context-dependent prognostic significance, with elevated GBP3 associated with increased mortality in brain glioma and lung squamous cell carcinoma but decreased risk in melanoma 5. Additionally, GBP3 participates in a STAT1-GBP3-STING feedback loop driving aortic inflammation and oxidative stress in acute aortic dissection 6. GBP3 expression is restricted to Simiiformes, likely originating from GBP1 gene duplication during primate evolution 7.

Sources cited
1
GBP3 governs caspase-4 activation on bacterial surfaces and triggers pyroptosis
PMID: 32541830
2
GBP3 directly binds bacterial lipopolysaccharide and localizes to intracellular microbes
PMID: 37567806
3
GBP3 promotes glioma cell proliferation via SQSTM1/p62-ERK1/2 signaling
PMID: 29128363
4
GBP3 enhances temozolomide resistance by stabilizing STING and upregulating DNA damage repair
PMID: 35780181
5
GBP3 has context-dependent prognostic significance across multiple cancer types
PMID: 37161254
6
GBP3 participates in STAT1-GBP3-STING feedback loop in acute aortic dissection
PMID: 40714274
7
GBP3 genes are present only in Simiiformes and likely originated from GBP1 duplication
PMID: 33550181
Disease Associationsⓘ20
bacterial diseaseOpen Targets
0.46Moderate
isolated agammaglobulinemiaOpen Targets
0.07Suggestive
Immunodeficiency due to a late component of complements deficiencyOpen Targets
0.06Suggestive
recurrent infections associated with rare immunoglobulin isotypes deficiencyOpen Targets
0.06Suggestive
Immunodeficiency by defective expression of HLA class 2Open Targets
0.05Suggestive
mannose-binding lectin deficiencyOpen Targets
0.05Suggestive
Autosomal recessive hyper-IgE syndromeOpen Targets
0.05Suggestive
Autosomal recessive hyper-IgE syndrome due to TYK2 deficiencyOpen Targets
0.05Suggestive
immunodeficiency 35Open Targets
0.05Suggestive
self-injurious ideationOpen Targets
0.05Suggestive
hyper-IgM syndrome type 2Open Targets
0.05Suggestive
X-linked agammaglobulinemiaOpen Targets
0.05Suggestive
gamma chain deficiencyOpen Targets
0.05Suggestive
MHC class II deficiencyOpen Targets
0.04Suggestive
immunodeficiency due to ficolin3 deficiencyOpen Targets
0.04Suggestive
severe combined immunodeficiency due to IKK2 deficiencyOpen Targets
0.04Suggestive
severe combined immunodeficiency, autosomal recessive, T cell-negative, B cell-negative, NK cell-positiveOpen Targets
0.04Suggestive
T-B+ severe combined immunodeficiency due to JAK3 deficiencyOpen Targets
0.04Suggestive
autosomal recessive severe congenital neutropenia due to JAGN1 deficiencyOpen Targets
0.04Suggestive
cancerOpen Targets
0.04Suggestive
Pathogenic Variants
No pathogenic variants reported on ClinVar for this gene.
View on ClinVar ↗
Related Genes
GBP2Protein interaction67%GBP1Protein interaction54%GBP5Shared pathway29%ACOD1Shared pathway20%GBP7Shared pathway20%GBP6Shared pathway20%
Tissue Expression6 tissues
Lung
100%
Ovary
51%
Liver
46%
Heart
42%
Bone Marrow
27%
Brain
16%
Gene Interaction Network
Click a node to explore
GBP3GBP2GBP1GBP5ACOD1GBP7GBP6
PROTEIN STRUCTURE
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AlphaFoldAI-predicted · UniProt Q9H0R5
View on AlphaFold ↗
Constraintⓘ
LOEUFⓘ
1.09LoF Tolerant
pLIⓘ
0.00Tolerant
Observed/Expected LoF0.87 [0.70–1.09]
RankingsWhere GBP3 stands among ~20K protein-coding genes
  • #13,421of 20,598
    Most Researched23
  • #11,146of 17,882
    Most Constrained (LOEUF)1.09
Genes detectedGBP3
Sources retrieved10 papers
Response time—
📄 Sources
10▼
1
Guanylate-binding proteins convert cytosolic bacteria into caspase-4 signaling platforms.
PMID: 32541830
Nat Immunol · 2020
1.00
2
Guanylate-binding proteins: mechanisms of pattern recognition and antimicrobial functions.
PMID: 37567806
Trends Biochem Sci · 2023
0.90
3
The function of guanylate binding protein 3 (GBP3) in human cancers by pan-cancer bioinformatics.
PMID: 37161254
Math Biosci Eng · 2023
0.80
4
A STAT1-GBP3-STING positive feedback loop governs inflammation, oxidative stress, and DNA damage to trigger acute aortic dissection.
PMID: 40714274
Cell Signal · 2025
0.70
5
GBP3 promotes glioma cell proliferation via SQSTM1/p62-ERK1/2 axis.
PMID: 29128363
Biochem Biophys Res Commun · 2018
0.60