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10 sources retrieved · Most recent: April 2026 · Index updated 15 days ago
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DIRAS3
DIRAS family GTPase 3
Chromosome 1 · 1p31.3
NCBI Gene: 9077Ensembl: ENSG00000162595.9HGNC: HGNC:687UniProt: O95661
75PubMed Papers
20Diseases
0Drugs
0Pathogenic Variants
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
protein bindingplasma membraneGTPase activitysmall GTPase-mediated signal transductionGenu valgumGenu varumiris disorderciliopathy
✦AI Summary

DIRAS3 is a maternally imprinted tumor suppressor gene encoding a 26 kDa GTPase with 60% homology to RAS and a distinctive 34-amino acid N-terminal extension 1. As a unique endogenous RAS inhibitor, DIRAS3 binds directly to RAS, disrupting RAS dimers and clusters to block RAS/MAPK signaling 2. DIRAS3 also inhibits PI3K/AKT and JAK/STAT pathways 1. Loss of DIRAS3 expression occurs frequently in ovarian, breast, lung, prostate, colon, brain, and thyroid cancers through methylation and deletion mechanisms 13. Reexpression of DIRAS3 inhibits cancer cell growth and motility while preventing angiogenesis 1. DIRAS3 is essential for autophagy induction through multiple mechanisms, including activation of the STK11/LKB1-PRKAA/AMPK pathway in KRAS-mutant cancers 4. DIRAS3 induces tumor dormancy in vivo and mediates ROS-dependent apoptosis in KRAS-driven pancreatic and ovarian carcinomas 14. Notably, fibronectin in the tumor microenvironment can inhibit DIRAS3-induced autophagy through integrin β1/FAK/AKT signaling 5. In high-grade gliomas, DIRAS3 upregulation paradoxically promotes immune evasion and poor prognosis 6. DIRAS3 expression in dormant, autophagic cancer cells serves as a prognostic biomarker and therapeutic target.

Sources cited
1
DIRAS3 is a maternally imprinted tumor suppressor gene encoding a 26 kDa GTPase with 60% homology to RAS and a distinctive 34-amino acid N-terminal extension .
PMID: 34667114
2
As a unique endogenous RAS inhibitor, DIRAS3 binds directly to RAS, disrupting RAS dimers and clusters to block RAS/MAPK signaling .
PMID: 31825828
3
DIRAS3 is essential for autophagy induction through multiple mechanisms, including activation of the STK11/LKB1-PRKAA/AMPK pathway in KRAS-mutant cancers .
PMID: 38169324
4
Notably, fibronectin in the tumor microenvironment can inhibit DIRAS3-induced autophagy through integrin β1/FAK/AKT signaling .
PMID: 40862729
5
In high-grade gliomas, DIRAS3 upregulation paradoxically promotes immune evasion and poor prognosis .
PMID: 40649981
Disease Associationsⓘ20
Genu valgumOpen Targets
0.14Weak
Genu varumOpen Targets
0.14Weak
iris disorderOpen Targets
0.14Weak
ciliopathyOpen Targets
0.12Weak
Crohn's diseaseOpen Targets
0.12Weak
placental retentionOpen Targets
0.11Weak
smoking initiationOpen Targets
0.10Weak
ovarian cancerOpen Targets
0.10Suggestive
gliomaOpen Targets
0.09Suggestive
non-small cell lung carcinomaOpen Targets
0.09Suggestive
gastric cancerOpen Targets
0.08Suggestive
thyroid cancerOpen Targets
0.08Suggestive
esophageal squamous cell carcinomaOpen Targets
0.08Suggestive
neoplasmOpen Targets
0.07Suggestive
breast cancerOpen Targets
0.06Suggestive
osteoporosisOpen Targets
0.05Suggestive
osteosarcomaOpen Targets
0.04Suggestive
lung carcinomaOpen Targets
0.04Suggestive
ovarian carcinomaOpen Targets
0.04Suggestive
adrenal gland pheochromocytomaOpen Targets
0.04Suggestive
Pathogenic Variants
No pathogenic variants reported on ClinVar for this gene.
View on ClinVar ↗
Related Genes
PIK3CBProtein interaction92%RAB42Shared pathway33%ZDBF2Shared pathway33%RALGPS2Shared pathway33%RASL12Shared pathway33%RASL10AShared pathway33%
Tissue Expression6 tissues
Ovary
100%
Heart
29%
Liver
11%
Brain
4%
Lung
3%
Bone Marrow
1%
Gene Interaction Network
Click a node to explore
DIRAS3PIK3CBRAB42ZDBF2RALGPS2RASL12RASL10A
PROTEIN STRUCTURE
Preparing viewer…
PDB6NAZ · 3.08 Å · X-ray
View on RCSB ↗
Constraintⓘ
LOEUFⓘ
1.84LoF Tolerant
pLIⓘ
0.34Tolerant
Observed/Expected LoF0.00 [0.00–1.84]
RankingsWhere DIRAS3 stands among ~20K protein-coding genes
  • #6,323of 20,598
    Most Researched75
  • #16,770of 17,882
    Most Constrained (LOEUF)1.84
Genes detectedDIRAS3
Sources retrieved10 papers
Response time—
📄 Sources
10▼
1
DIRAS3: An Imprinted Tumor Suppressor Gene that Regulates RAS and PI3K-driven Cancer Growth, Motility, Autophagy, and Tumor Dormancy.
PMID: 34667114
Mol Cancer Ther · 2022
1.00
2
DIRAS3 induces autophagy and enhances sensitivity to anti-autophagic therapy in KRAS-driven pancreatic and ovarian carcinomas.
PMID: 38169324
Autophagy · 2024
0.90
3
BST2 and DIRAS3 Drive Immune Evasion and Tumor Progression in High-Grade Glioma.
PMID: 40649981
Int J Mol Sci · 2025
0.80
4
Frequent biallelic inactivation and transcriptional silencing of the DIRAS3 gene at 1p31 in oligodendroglial tumors with 1p loss.
PMID: 18302158
Int J Cancer · 2008
0.70
5
DIRAS3 Inhibits Ovarian Cancer Cell Growth by Blocking the Fibronectin-Mediated Integrin β1/FAK/AKT Signaling Pathway.
PMID: 40862729
Cells · 2025
0.60