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10 sources retrieved · Most recent: April 2026 · Index updated 14 days ago
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EYA3
EYA transcriptional coactivator and phosphatase 3
Chromosome 1 · 1p35.3
NCBI Gene: 2140Ensembl: ENSG00000158161.17HGNC: HGNC:3521UniProt: Q99504
50PubMed Papers
20Diseases
0Drugs
0Pathogenic Variants
FUNCTIONAL ROLE
DNA RepairTranscription Factor
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
protein tyrosine phosphatase activityprotein bindinghistone H2AXY142 phosphatase activitydouble-strand break repairGenu valgumGenu varumneoplasmtriple-negative breast cancer
✦AI Summary

EYA3 is a multifunctional tyrosine phosphatase and transcriptional coactivator with roles in DNA repair, transcriptional regulation, and disease pathogenesis. Biochemically, EYA3 functions as a tyrosine phosphatase that specifically dephosphorylates H2AX at Tyr-142, promoting efficient DNA repair and recruitment of repair complexes 1. Additionally, EYA3 recruits PP2A-B55α to dephosphorylate Myc at Thr-58, stabilizing Myc and enhancing cell proliferation 23. EYA3 serves as a transcriptional coactivator of SIX family proteins, with its activity regulated by alternative splicing during myogenesis through RBFOX2-mediated control 4. EYA3 identifies WDR1 as a tyrosine phosphatase substrate, regulating actin cytoskeletal reorganization 5. Disease relevance is substantial: EYA3 promotes triple-negative breast cancer progression by stabilizing Myc and suppressing NK cell infiltration into the premetastatic niche via NF-κB/CCL2 signaling 6. In Ewing sarcoma, EYA3 promotes tumor growth and angiogenesis through VEGFA regulation and DNA damage repair 1. EYA3 elevation in pulmonary arterial hypertension promotes vascular remodeling by enhancing smooth muscle cell survival under DNA damage 7. Therapeutically, EYA3 tyrosine phosphatase inhibition represents a viable strategy across multiple cancer types and pulmonary hypertension.

Sources cited
1
Biochemically, EYA3 functions as a tyrosine phosphatase that specifically dephosphorylates H2AX at Tyr-142, promoting efficient DNA repair and recruitment of repair complexes .
PMID: 33649104
2
EYA3 serves as a transcriptional coactivator of SIX family proteins, with its activity regulated by alternative splicing during myogenesis through RBFOX2-mediated control .
PMID: 38026174
3
EYA3 identifies WDR1 as a tyrosine phosphatase substrate, regulating actin cytoskeletal reorganization .
PMID: 29440662
4
Disease relevance is substantial: EYA3 promotes triple-negative breast cancer progression by stabilizing Myc and suppressing NK cell infiltration into the premetastatic niche via NF-κB/CCL2 signaling .
PMID: 40333987
5
EYA3 elevation in pulmonary arterial hypertension promotes vascular remodeling by enhancing smooth muscle cell survival under DNA damage .
PMID: 31515519
Disease Associationsⓘ20
Genu valgumOpen Targets
0.15Weak
Genu varumOpen Targets
0.14Weak
neoplasmOpen Targets
0.09Suggestive
triple-negative breast cancerOpen Targets
0.08Suggestive
colorectal carcinomaOpen Targets
0.07Suggestive
Abnormality of the immune systemOpen Targets
0.06Suggestive
Romano-Ward syndromeOpen Targets
0.06Suggestive
Familial progressive cardiac conduction defectOpen Targets
0.05Suggestive
Brugada syndromeOpen Targets
0.05Suggestive
Arrhythmogenic right ventricular dysplasiaOpen Targets
0.05Suggestive
Familial short QT syndromeOpen Targets
0.05Suggestive
familial atrial fibrillationOpen Targets
0.05Suggestive
hypertrophic cardiomyopathyOpen Targets
0.05Suggestive
breast cancerOpen Targets
0.05Suggestive
Rare familial disorder with hypertrophic cardiomyopathyOpen Targets
0.05Suggestive
atrial fibrillationOpen Targets
0.04Suggestive
dilated cardiomyopathyOpen Targets
0.04Suggestive
familial sick sinus syndromeOpen Targets
0.04Suggestive
Atrial stand stillOpen Targets
0.04Suggestive
Wolff-Parkinson-White SyndromeOpen Targets
0.04Suggestive
Pathogenic Variants
No pathogenic variants reported on ClinVar for this gene.
View on ClinVar ↗
Related Genes
SIX1Protein interaction91%SIX2Protein interaction88%SIX5Protein interaction83%DACH1Protein interaction75%SIX4Protein interaction75%EYA1Shared pathway70%
Tissue Expression6 tissues
Heart
100%
Brain
94%
Lung
92%
Liver
78%
Ovary
74%
Bone Marrow
59%
Gene Interaction Network
Click a node to explore
EYA3SIX1SIX2SIX5DACH1SIX4EYA1
PROTEIN STRUCTURE
Preparing viewer…
PDB9C7T · 2.70 Å · EM
View on RCSB ↗
Constraintⓘ
LOEUFⓘ
0.61LoF Tolerant
pLIⓘ
0.12Tolerant
Observed/Expected LoF0.43 [0.30–0.61]
RankingsWhere EYA3 stands among ~20K protein-coding genes
  • #8,806of 20,598
    Most Researched50
  • #4,224of 17,882
    Most Constrained (LOEUF)0.61 · top quartile
Genes detectedEYA3
Sources retrieved10 papers
Response time—
📄 Sources
10▼
1
Cryo-EM structures reveal the PP2A-B55α and Eya3 interaction that can be disrupted by a peptide inhibitor.
PMID: 40414499
J Biol Chem · 2025
1.00
2
EYA3 regulation of NF-κB and CCL2 suppresses cytotoxic NK cells in the premetastatic niche to promote TNBC metastasis.
PMID: 40333987
Sci Adv · 2025
0.90
3
Cryo-EM structures of PP2A:B55 with p107 and Eya3 define substrate recruitment.
PMID: 40247147
Nat Struct Mol Biol · 2025
0.80
4
Targeting EYA3 in Ewing Sarcoma Retards Tumor Growth and Angiogenesis.
PMID: 33649104
Mol Cancer Ther · 2021
0.70
5
RBFOX2 regulated EYA3 isoforms partner with SIX4 or ZBTB1 to control transcription during myogenesis.
PMID: 38026174
iScience · 2023
0.60