4 sources retrieved · Most recent: April 2026 · Index updated 15 days ago
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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
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.
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: 394813782
IRGQ regulates MHC class I quality control through autophagic degradation; lower IRGQ levels associated with improved HCC survival and enhanced T-cell immunity
PMID: 414499733
ESCO1 acetyltransferase acetylates IRGQ and promotes its ubiquitin-proteasome degradation in cervical cancer
PMID: 398365024
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.
Familial prostate cancerOpen Targets
prostate cancerOpen Targets
Romano-Ward syndromeOpen Targets
Familial progressive cardiac conduction defectOpen Targets
familial atrial fibrillationOpen Targets
Familial short QT syndromeOpen Targets
catecholaminergic polymorphic ventricular tachycardiaOpen Targets
Brugada syndromeOpen Targets
Arrhythmogenic right ventricular dysplasiaOpen Targets
hypertrophic cardiomyopathyOpen Targets
Rare familial disorder with hypertrophic cardiomyopathyOpen Targets
atrial fibrillationOpen Targets
sudden cardiac arrestOpen Targets
Idiopathic ventricular fibrillation, not Brugada typeOpen Targets
dilated cardiomyopathyOpen Targets
sinoatrial node dysfunction and deafnessOpen Targets
Atrial stand stillOpen Targets
cardiac conduction defectOpen Targets
long QT syndrome 9Open Targets
long QT syndrome 5Open Targets
No pathogenic variants reported on ClinVar for this gene.