HomeAboutRankingsData Sources
© 2026 GeneE
🧬
GeneE
4 sources retrieved · Most recent: April 2026 · Index updated 15 days ago
ⓘGeneE is for informational purposes only. It is not a substitute for professional medical advice, diagnosis, or treatment.
IRGQ
immunity related GTPase Q
Chromosome 19 · 19q13.31
NCBI Gene: 126298Ensembl: ENSG00000167378.11HGNC: HGNC:24868UniProt: Q8WZA9
30PubMed Papers
20Diseases
0Drugs
0Pathogenic Variants
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
GTPase activityGTP bindingprotein bindingprotein-macromolecule adaptor activityprostate cancerFamilial prostate cancerRomano-Ward syndromeFamilial progressive cardiac conduction defect
✦AI Summary

IRGQ is an autophagy receptor that functions in MHC class I quality control and immune regulation 1. Unlike other immunity-related GTPases, IRGQ lacks GTPase activity but serves as a molecular adapter that specifically recognizes ubiquitinated misfolded MHC class I molecules and recruits them to autophagy machinery through binding to GABARAPL2 and LC3B 1. This interaction directs misfolded MHC class I toward lysosomal degradation, preventing accumulation of defective complexes at the cell surface 1. IRGQ-mediated degradation suppresses CD8+ T-cell responses by reducing surface presentation of aberrant MHC class I heavy chains, thereby promoting tumor immune evasion 12. In hepatocellular carcinoma, reduced IRGQ expression correlates with improved patient survival and enhanced anti-tumor immunity 12. IRGQ is regulated by the acetyltransferase ESCO1, which acetylates IRGQ and facilitates its ubiquitin-proteasome-mediated degradation in HPV-induced cervical cancer 3. These findings identify IRGQ as an immunomodulatory target for enhancing anti-tumor immunity through therapeutic inhibition 4.

Sources cited
1
IRGQ functions as an autophagy receptor for misfolded MHC class I molecules, binding GABARAPL2/LC3B and directing cargo to lysosomal degradation; lacks GTPase activity; IRGQ knockout increases surface MHC class I and improves survival in HCC
PMID: 39481378
2
IRGQ regulates MHC class I quality control through autophagic degradation; lower IRGQ levels associated with improved HCC survival and enhanced T-cell immunity
PMID: 41449973
3
ESCO1 acetyltransferase acetylates IRGQ and promotes its ubiquitin-proteasome degradation in cervical cancer
PMID: 39836502
4
IRGQ identified as a selective autophagy receptor facilitating immune evasion in HCC by degrading MHC-I molecules
PMID: 39627113
⚠Limited data available — This gene has 4 indexed publications. Summary and analysis may be incomplete.
Disease Associationsⓘ20
Familial prostate cancerOpen Targets
0.11Weak
prostate cancerOpen Targets
0.11Weak
Romano-Ward syndromeOpen Targets
0.08Suggestive
Familial progressive cardiac conduction defectOpen Targets
0.08Suggestive
familial atrial fibrillationOpen Targets
0.07Suggestive
Familial short QT syndromeOpen Targets
0.07Suggestive
catecholaminergic polymorphic ventricular tachycardiaOpen Targets
0.07Suggestive
Brugada syndromeOpen Targets
0.07Suggestive
Arrhythmogenic right ventricular dysplasiaOpen Targets
0.07Suggestive
hypertrophic cardiomyopathyOpen Targets
0.07Suggestive
Rare familial disorder with hypertrophic cardiomyopathyOpen Targets
0.07Suggestive
atrial fibrillationOpen Targets
0.06Suggestive
sudden cardiac arrestOpen Targets
0.06Suggestive
Idiopathic ventricular fibrillation, not Brugada typeOpen Targets
0.06Suggestive
dilated cardiomyopathyOpen Targets
0.06Suggestive
sinoatrial node dysfunction and deafnessOpen Targets
0.06Suggestive
Atrial stand stillOpen Targets
0.05Suggestive
cardiac conduction defectOpen Targets
0.05Suggestive
long QT syndrome 9Open Targets
0.05Suggestive
long QT syndrome 5Open Targets
0.05Suggestive
Pathogenic Variants
No pathogenic variants reported on ClinVar for this gene.
View on ClinVar ↗
Related Genes
RETREG2Shared pathway33%SAYSD1Shared pathway25%SLC35D3Shared pathway25%RAB37Shared pathway25%DHRSXShared pathway25%ZSWIM8Shared pathway25%
Tissue Expression6 tissues
Bone Marrow
100%
Brain
76%
Ovary
63%
Heart
60%
Lung
50%
Liver
48%
Gene Interaction Network
Click a node to explore
IRGQRETREG2SAYSD1SLC35D3RAB37DHRSXZSWIM8
PROTEIN STRUCTURE
Preparing viewer…
PDB8Q7K · 1.60 Å · X-ray
View on RCSB ↗
Constraintⓘ
LOEUFⓘ
1.06LoF Tolerant
pLIⓘ
0.00Tolerant
Observed/Expected LoF0.68 [0.45–1.06]
RankingsWhere IRGQ stands among ~20K protein-coding genes
  • #11,949of 20,598
    Most Researched30
  • #10,627of 17,882
    Most Constrained (LOEUF)1.06
Genes detectedIRGQ
Sources retrieved4 papers
Response time—
📄 Sources
4
1
IRGQ-mediated autophagy in MHC class I quality control promotes tumor immune evasion.
PMID: 39481378
Cell · 2024
1.00
2
Autophagy-mediated quality control of MHC class I molecules regulates tumor evasion.
PMID: 41449973
Autophagy · 2026
0.75
3
HPV E6/E7-Induced Acetylation of a Peptide Encoded by a Long Non-Coding RNA Inhibits Ferroptosis to Promote the Malignancy of Cervical Cancer.
PMID: 39836502
Adv Sci (Weinh) · 2025
0.50
4
Selective autophagy receptor hinders antitumor immunity.
PMID: 39627113
Trends Cancer · 2025
0.25