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10 sources retrieved · Most recent: April 2026 · Index updated 15 days ago
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PREX2
phosphatidylinositol-3,4,5-trisphosphate dependent Rac exchange factor 2
Chromosome 8 · 8q13.2
NCBI Gene: 80243Ensembl: ENSG00000046889.20HGNC: HGNC:22950UniProt: Q70Z35
39PubMed Papers
20Diseases
0Drugs
1Pathogenic Variants
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
guanyl-nucleotide exchange factor activityG protein-coupled receptor signaling pathwayGTPase activator activityprotein bindingskin basal cell carcinomacutaneous melanomapancreatic ductal adenocarcinomalung carcinoma
✦AI Summary

PREX2 is a guanine nucleotide exchange factor (GEF) that activates RAC1 by promoting GDP-to-GTP exchange, functioning downstream of both G protein-coupled receptors and phosphoinositide 3-kinase (PI3K) 1. Its activity is synergistically enhanced by phosphatidylinositol 3,4,5-trisphosphate and G protein βγ subunits, with mechanistic involvement in the PI3K/AKT signaling pathway 12. PREX2 interacts with PTEN, regulating its protein stability and activity; truncating mutations abolish PTEN binding while activating RAC1 GEF activity 1. Clinically, PREX2 exhibits oncogenic roles across multiple cancer types. In pancreatic ductal adenocarcinoma, PREX2 gene disruption occurs as a driver of carcinogenesis 3. In colorectal cancer, PREX2 overexpression confers radiation resistance by suppressing immunogenic cell death through the cGAS/STING/IFN pathway and promoting DNA repair 4. In NRAS-mutant melanoma, truncating PREX2 mutations promote tumorigenesis by deregulating cell cycle and cytoskeleton genes through epigenetic mechanisms, including CDKN1C downregulation 12. In non-small cell lung cancer, PREX2 promotes tumor growth through interaction with AHCYL1, which enhances its GEF activity 5. In BRAF-mutant melanoma, the PREX2/RAC1/PI3Kβ axis drives therapeutic resistance, and dual targeting of MAPK and this pathway shows superior efficacy 6. PREX2 mutations in hepatocellular carcinoma, particularly S1113R, increase protein stability and promote aggressive behavior 7.

Sources cited
1
PREX2 acts as a RAC1 GEF; truncating mutations activate GEF activity, abolish PTEN binding, activate PI3K/AKT signaling, and regulate CDKN1C expression
PMID: 26884185
2
Reciprocal PTEN-PREX2 regulation; PI3K/AKT inhibition destabilizes PREX2; PREX2 mutations alter histone marks and DNA methylation affecting p57/CDKN1C and IGF2 expression
PMID: 27111337
3
PREX2 identified as new candidate driver of pancreatic ductal adenocarcinoma through chromosomal rearrangements and gene disruption
PMID: 25719666
4
PREX2 upregulation in radioresistant colorectal cancer cells; promotes DNA repair and radiation resistance by suppressing cGAS/STING/IFN pathway and CD8+ T cell infiltration
PMID: 38609982
5
PREX2 promotes NSCLC growth; AHCYL1 enhances PREX2 GEF activity by alleviating mutual inhibition with PTEN
PMID: 40365293
6
PREX2/RAC1/PI3Kβ axis drives BRAF-inhibitor resistance in melanoma; dual pathway targeting shows superior therapeutic efficacy
PMID: 39636745
7
PREX2 S1113R mutation in hepatocellular carcinoma increases protein stability, promotes cell proliferation and migration, and impairs ubiquitin-mediated degradation
PMID: 30796242
Disease Associationsⓘ20
cutaneous melanomaOpen Targets
0.37Weak
skin basal cell carcinomaOpen Targets
0.37Weak
pancreatic ductal adenocarcinomaOpen Targets
0.37Weak
lung carcinomaOpen Targets
0.37Weak
Angiomatous MeningiomaOpen Targets
0.37Weak
Atypical MeningiomaOpen Targets
0.37Weak
bile duct carcinomaOpen Targets
0.37Weak
breast ductal adenocarcinomaOpen Targets
0.37Weak
colorectal adenocarcinomaOpen Targets
0.37Weak
esophageal adenocarcinomaOpen Targets
0.37Weak
gastric carcinomaOpen Targets
0.37Weak
HER2 Positive Breast CarcinomaOpen Targets
0.37Weak
kidney neoplasmOpen Targets
0.37Weak
lymphoid neoplasmOpen Targets
0.37Weak
Meningothelial MeningiomaOpen Targets
0.37Weak
Merkel cell skin cancerOpen Targets
0.37Weak
nodular melanomaOpen Targets
0.37Weak
pancreatic carcinomaOpen Targets
0.37Weak
superficial spreading melanomaOpen Targets
0.37Weak
Transitional MeningiomaOpen Targets
0.37Weak
Pathogenic Variants1
NM_024870.4(PREX2):c.3355G>A (p.Ala1119Thr)Likely pathogenic
Cerebral arteriovenous malformation
★☆☆☆2018→ Residue 1119
View on ClinVar ↗
Related Genes
PTENProtein interaction99%CDC42Protein interaction85%RHOAProtein interaction79%RAC1Protein interaction78%MCF2LShared pathway67%DNMBPShared pathway40%
Tissue Expression6 tissues
Heart
100%
Brain
52%
Lung
41%
Ovary
28%
Bone Marrow
15%
Liver
11%
Gene Interaction Network
Click a node to explore
PREX2PTENCDC42RHOARAC1MCF2LDNMBP
PROTEIN STRUCTURE
Preparing viewer…
PDB6BNM · 1.90 Å · X-ray
View on RCSB ↗
Constraintⓘ
LOEUFⓘ
0.65LoF Tolerant
pLIⓘ
0.00Tolerant
Observed/Expected LoF0.52 [0.42–0.65]
RankingsWhere PREX2 stands among ~20K protein-coding genes
  • #10,379of 20,598
    Most Researched39
  • #5,181of 5,498
    Most Pathogenic Variants1
  • #4,733of 17,882
    Most Constrained (LOEUF)0.65
Genes detectedPREX2
Sources retrieved10 papers
Response time—
📄 Sources
10▼
1
Whole genomes redefine the mutational landscape of pancreatic cancer.
PMID: 25719666
Nature · 2015
1.00
2
PREX2 contributes to radiation resistance by inhibiting radiotherapy-induced tumor immunogenicity via cGAS/STING/IFNs pathway in colorectal cancer.
PMID: 38609982
BMC Med · 2024
0.90
3
AHCYL1 mediates the tumor-promoting effect of PREX2 in non-small cell lung carcinoma.
PMID: 40365293
Theranostics · 2025
0.80
4
Targeting the PREX2/RAC1/PI3Kβ Signaling Axis Confers Sensitivity to Clinically Relevant Therapeutic Approaches in Melanoma.
PMID: 39636745
Cancer Res · 2025
0.70
5
Truncating PREX2 mutations activate its GEF activity and alter gene expression regulation in NRAS-mutant melanoma.
PMID: 26884185
Proc Natl Acad Sci U S A · 2016
0.60