HomeAboutRankingsData Sources
© 2026 GeneE
🧬
GeneE
10 sources retrieved · Most recent: April 2026 · Index updated 14 days ago
ⓘGeneE is for informational purposes only. It is not a substitute for professional medical advice, diagnosis, or treatment.
GBP1
guanylate binding protein 1
Chromosome 1 · 1p22.2
NCBI Gene: 2633Ensembl: ENSG00000117228.12HGNC: HGNC:4182UniProt: P32455
129PubMed Papers
20Diseases
0Drugs
1Pathogenic Variants
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
cytoplasmic vesicleactin bindingGTPase activityG protein activitybacterial diseaseinfluenzaneurodegenerative diseaseNeutrophil inclusion bodies
✦AI Summary

GBP1 is an interferon-inducible GTPase that functions as a critical antimicrobial effector in cell-autonomous immunity 1. Its primary function involves hydrolysis of GTP to GMP through two consecutive cleavage reactions, converting GTP to GDP and then to GMP in a processive manner 2. Following pathogen infection, GBP1 localizes to pathogen-containing vacuoles and cytosol-escaped bacteria, where it executes multiple defense mechanisms 34. Mechanistically, GBP1 forms polyvalent protein coats that encapsulate Gram-negative bacteria and directly binds lipopolysaccharide (LPS), disrupting the O-antigen barrier to expose cryptic lipid A that activates the caspase-4 inflammasome 256. This assembly constitutes a massive host-defense nanomachine comprising approximately 30,000 GBP1 molecules with integrated caspase-4 and gasdermin D 7. GBP1 also promotes inflammasome assembly against intracellular pathogens like Legionella and Toxoplasma by liberating pathogenic molecules into the cytosol, where they trigger both apoptosis and pyroptosis 48. Physiologically, GBP1 requires tight regulation; uncontrolled expression induces cytotoxic Golgi fragmentation, prevented by PIM1-mediated phosphorylation and 14-3-3σ sequestration 9. In disease, GBP1 overexpression in immune-hot IDH-mutant astrocytomas correlates with worse prognosis 10. GBP1 confers protection against bacterial pathogens (L.monocytogenes, M.bovis BCG), protozoan parasites (T.gondii), and exhibits antiviral activity 411.

Sources cited
1
GBP1 is an interferon-inducible GTPase that functions as a critical antimicrobial effector in cell-autonomous immunity .
PMID: 33314740
2
Its primary function involves hydrolysis of GTP to GMP through two consecutive cleavage reactions, converting GTP to GDP and then to GMP in a processive manner .
PMID: 32510692
3
This assembly constitutes a massive host-defense nanomachine comprising approximately 30,000 GBP1 molecules with integrated caspase-4 and gasdermin D .
PMID: 38422126
4
Physiologically, GBP1 requires tight regulation; uncontrolled expression induces cytotoxic Golgi fragmentation, prevented by PIM1-mediated phosphorylation and 14-3-3σ sequestration .
PMID: 37797010
5
In disease, GBP1 overexpression in immune-hot IDH-mutant astrocytomas correlates with worse prognosis .
PMID: 40939590
Disease Associationsⓘ20
bacterial diseaseOpen Targets
0.53Moderate
influenzaOpen Targets
0.37Weak
neurodegenerative diseaseOpen Targets
0.30Weak
Neutrophil inclusion bodiesOpen Targets
0.26Weak
muscular diseaseOpen Targets
0.26Weak
neoplasmOpen Targets
0.11Weak
glioblastoma multiformeOpen Targets
0.11Weak
breast cancerOpen Targets
0.10Weak
lung adenocarcinomaOpen Targets
0.10Suggestive
cancerOpen Targets
0.10Suggestive
infectionOpen Targets
0.09Suggestive
cervical cancerOpen Targets
0.09Suggestive
triple-negative breast cancerOpen Targets
0.08Suggestive
gliomaOpen Targets
0.08Suggestive
lung cancerOpen Targets
0.08Suggestive
Townes-Brocks syndromeOpen Targets
0.07Suggestive
MODYOpen Targets
0.07Suggestive
isolated agammaglobulinemiaOpen Targets
0.07Suggestive
hepatitis B virus infectionOpen Targets
0.07Suggestive
prostate cancerOpen Targets
0.07Suggestive
Pathogenic Variants1
NM_002053.3(GBP1):c.1665+30_1665+35delLikely pathogenic
Neutrophil inclusion bodies
☆☆☆☆
View on ClinVar ↗
Related Genes
OAS2Protein interaction100%OAS1Protein interaction99%MX1Protein interaction97%IFIT1Protein interaction97%STAT1Protein interaction96%CXCL10Protein interaction96%
Tissue Expression6 tissues
Lung
100%
Liver
82%
Ovary
44%
Heart
27%
Brain
19%
Bone Marrow
15%
Gene Interaction Network
Click a node to explore
GBP1OAS2OAS1MX1IFIT1STAT1CXCL10
PROTEIN STRUCTURE
Preparing viewer…
PDB1F5N · 1.70 Å · X-ray
View on RCSB ↗
Constraintⓘ
LOEUFⓘ
1.12LoF Tolerant
pLIⓘ
0.00Tolerant
Observed/Expected LoF0.91 [0.74–1.12]
RankingsWhere GBP1 stands among ~20K protein-coding genes
  • #3,619of 20,598
    Most Researched129 · top quartile
  • #4,809of 5,498
    Most Pathogenic Variants1
  • #11,521of 17,882
    Most Constrained (LOEUF)1.12
Genes detectedGBP1
Sources retrieved10 papers
Response time—
📄 Sources
10▼
1
PIM1 controls GBP1 activity to limit self-damage and to guard against pathogen infection.
PMID: 37797010
Science · 2023
1.00
2
Native architecture of a human GBP1 defense complex for cell-autonomous immunity to infection.
PMID: 38422126
Science · 2024
0.90
3
Protein-based classification reveals an immune-hot subtype in IDH mutant astrocytoma with worse prognosis.
PMID: 40939590
Cancer Cell · 2025
0.80
4
Human GBP1 facilitates the rupture of the
PMID: 37737612
mBio · 2023
0.70
5
LPS-aggregating proteins GBP1 and GBP2 are each sufficient to enhance caspase-4 activation both in cellulo and in vitro.
PMID: 37023136
Proc Natl Acad Sci U S A · 2023
0.60