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GeneE
25 sources retrieved Β· Most recent: April 2026 Β· Index updated 14 days ago
β“˜GeneE is for informational purposes only. It is not a substitute for professional medical advice, diagnosis, or treatment.
TEK
TEK receptor tyrosine kinase
Chromosome 9 Β· 9p21.2
NCBI Gene: 7010Ensembl: ENSG00000120156.23HGNC: HGNC:11724UniProt: Q02763
304PubMed Papers
22Diseases
7Drugs
61Pathogenic Variants
FUNCTIONAL ROLE
Hub GeneKinaseReceptor
RESEARCH IMPACT
TrendingVariant-Rich
CLINICAL
FDA Approved TargetOMIM Disease Gene
DATA QUALITY
βœ“ Experimental GO Evidenceβœ“ Swiss-Prot Reviewed
Tie signaling pathwaypositive regulation of MAPK cascadepositive regulation of angiogenesisactin filamentmultiple cutaneous and mucosal venous malformationsMucocutaneous venous malformationscongenital glaucomametastatic colorectal cancer
✦AI Summary

TEK (TEK receptor tyrosine kinase) is a cell-surface receptor tyrosine kinase that functions as the primary mediator of angiopoietin signaling in vascular biology. TEK binds angiopoietins (ANGPT1, ANGPT2, and ANGPT4) to regulate critical endothelial cell processes including angiogenesis, survival, migration, and adhesion [31108880]. The receptor operates through context-dependent signaling: in quiescent vessels, ANGPT1-TEK activation preferentially engages phosphatidylinositol 3-kinase and AKT cascades to promote vascular stability, while in migrating endothelial cells, it activates focal adhesion complexes and MAPK signaling to stimulate sprouting angiogenesis [31108880]. TEK expression increases during tumor angiogenesis, with stalk cells transitioning from high chemokine expression to elevated TEK as neovascularization progresses through angiogenic stages [38987599]. Beyond angiogenesis, TEK maintains vascular integrity and exerts anti-inflammatory effects by preventing leakage of plasma proteins and leukocytes from blood vessels [31108880]. Clinically, TEK is implicated in venous malformations through somatic gain-of-function mutations, which cause excessive signaling through the PI3K-AKT-mTOR pathway [38880808]. Recent studies reveal a PI3K-FOXO1-ANGPT-TEK feedforward circuit driving PIK3CA-related venous malformations, where PI3KΞ± hyperactivity amplifies upstream TIE2 receptor signaling [40410415]. TEK inhibition and ANGPT antagonism show promise as therapeutic targets for these previously intractable vascular anomalies [40410415].

Sources cited
1
TEK is a tyrosine kinase receptor for angiopoietins that mediates angiogenesis and vascular stability through context-dependent signaling pathways
PMID: 31108880
2
TEK expression increases in stalk cells during tumor angiogenesis progression through angiogenic stages
PMID: 38987599
3
TEK somatic gain-of-function mutations cause venous malformations through PI3K-AKT-mTOR pathway hyperactivation, and PI3KΞ± inhibition improves established TEK-related malformations in patients
PMID: 38880808
4
A PI3K-FOXO1-ANGPT-TEK feedforward circuit drives PIK3CA-related venous malformations, and TEK inhibition suppresses their growth
PMID: 40410415
⚠Limited data available β€” This gene has 4 indexed publications. Summary and analysis may be incomplete.
Disease Associationsβ“˜22
multiple cutaneous and mucosal venous malformationsOpen Targets
0.81Strong
Mucocutaneous venous malformationsOpen Targets
0.76Strong
congenital glaucomaOpen Targets
0.73Strong
metastatic colorectal cancerOpen Targets
0.55Moderate
colorectal cancerOpen Targets
0.54Moderate
vascular malformationOpen Targets
0.53Moderate
medullary thyroid gland carcinomaOpen Targets
0.52Moderate
neoplasmOpen Targets
0.51Moderate
Abnormal cardiovascular system morphologyOpen Targets
0.51Moderate
Venous malformationOpen Targets
0.50Moderate
colorectal neoplasmOpen Targets
0.49Moderate
blue rubber bleb nevusOpen Targets
0.41Moderate
non-small cell lung carcinomaOpen Targets
0.39Weak
thyroid neoplasmOpen Targets
0.39Weak
hepatocellular carcinomaOpen Targets
0.39Weak
primary congenital glaucomaOpen Targets
0.37Weak
thyroid carcinomaOpen Targets
0.37Weak
gastrointestinal stromal tumorOpen Targets
0.35Weak
lung cancerOpen Targets
0.35Weak
ventricular septal defectOpen Targets
0.34Weak
Dominantly inherited venous malformationsUniProt
Glaucoma 3, primary congenital, EUniProt
Pathogenic Variants61
NM_000459.5(TEK):c.2744G>A (p.Arg915His)Pathogenic
Multiple cutaneous and mucosal venous malformations|not provided|Vascular skin disorders
β˜…β˜…β˜†β˜†2025β†’ Residue 915
NM_000459.5(TEK):c.3295C>T (p.Arg1099Ter)Pathogenic
Abnormal cardiovascular system morphology|Multiple cutaneous and mucosal venous malformations
β˜…β˜…β˜†β˜†2025β†’ Residue 1099
NM_000459.5(TEK):c.2743C>T (p.Arg915Cys)Pathogenic
Abnormal cardiovascular system morphology|not provided|Multiple cutaneous and mucosal venous malformations|Vascular malformation
β˜…β˜…β˜†β˜†2025β†’ Residue 915
NM_000459.5(TEK):c.2690A>G (p.Tyr897Cys)Pathogenic
Multiple cutaneous and mucosal venous malformations|not provided|Abnormal cardiovascular system morphology|Blue rubber bleb nevus;Ventricular septal defect
β˜…β˜…β˜†β˜†2025β†’ Residue 897
NM_000459.5(TEK):c.1552C>T (p.Gln518Ter)Pathogenic
Glaucoma 3, primary congenital, E;Glaucoma 3, primary infantile, B;Multiple cutaneous and mucosal venous malformations|not provided
β˜…β˜…β˜†β˜†2025β†’ Residue 518
NM_000459.5(TEK):c.2752C>T (p.Arg918Cys)Pathogenic
Abnormal cardiovascular system morphology|Multiple cutaneous and mucosal venous malformations|not provided
β˜…β˜…β˜†β˜†2024β†’ Residue 918
NM_000459.5(TEK):c.2545C>T (p.Arg849Trp)Pathogenic
Multiple cutaneous and mucosal venous malformations|Abnormal cardiovascular system morphology|Segmental undergrowth associated with venous malformation without capillary component|not provided|TEK-related disorder
β˜…β˜…β˜†β˜†2024β†’ Residue 849
NM_000459.5(TEK):c.2689T>A (p.Tyr897Asn)Pathogenic
not provided|Multiple cutaneous and mucosal venous malformations
β˜…β˜…β˜†β˜†2024β†’ Residue 897
NM_000459.5(TEK):c.3314_3316delinsACC (p.Thr1105_Thr1106delinsAsnPro)Pathogenic
not provided|Vascular malformation
β˜…β˜…β˜†β˜†2024β†’ Residue 1105
NM_000459.5(TEK):c.3325G>T (p.Glu1109Ter)Pathogenic
Segmental undergrowth associated with venous malformation without capillary component|not provided|Vascular malformation
β˜…β˜…β˜†β˜†2023β†’ Residue 1109
NM_000459.5(TEK):c.2740C>T (p.Leu914Phe)Pathogenic
Bockenheimer syndrome|not provided|Abnormal cardiovascular system morphology|Vascular malformation
β˜…β˜…β˜†β˜†2023β†’ Residue 914
NM_000459.5(TEK):c.2689T>C (p.Tyr897His)Pathogenic
not provided|Vascular malformation
β˜…β˜…β˜†β˜†2023β†’ Residue 897
NM_000459.5(TEK):c.3327_3328del (p.Lys1110fs)Likely pathogenic
Vascular malformation
β˜…β˜†β˜†β˜†2025β†’ Residue 1110
NM_000459.5(TEK):c.1284del (p.Val429fs)Likely pathogenic
Glaucoma 3, primary congenital, E
β˜…β˜†β˜†β˜†2025β†’ Residue 429
NM_000459.5(TEK):c.3330_3334del (p.Lys1110fs)Likely pathogenic
Venous malformation
β˜…β˜†β˜†β˜†2025β†’ Residue 1110
NM_000459.5(TEK):c.2690A>T (p.Tyr897Phe)Pathogenic
Venous malformation
β˜…β˜†β˜†β˜†2025β†’ Residue 897
NM_000459.5(TEK):c.3324_3334del (p.Glu1109fs)Likely pathogenic
Multiple cutaneous and mucosal venous malformations
β˜…β˜†β˜†β˜†2025β†’ Residue 1109
NM_000459.5(TEK):c.3330_3335delinsT (p.Lys1110fs)Likely pathogenic
Venous malformation
β˜…β˜†β˜†β˜†2025β†’ Residue 1110
NM_000459.5(TEK):c.3103G>C (p.Gly1035Arg)Pathogenic
not provided
β˜…β˜†β˜†β˜†2025β†’ Residue 1035
NM_000459.5(TEK):c.889C>T (p.Gln297Ter)Likely pathogenic
not provided
β˜…β˜†β˜†β˜†2025β†’ Residue 297
View on ClinVar β†—
Drug Targets7
ALTIRATINIBPhase I
Hepatocyte growth factor receptor inhibitor
CEP-11981Phase I
Vascular endothelial growth factor receptor inhibitor
FORETINIBPhase II
Stem cell growth factor receptor inhibitor
breast cancer
GLESATINIBPhase II
Macrophage-stimulating protein receptor inhibitor
non-small cell lung carcinoma
PEXMETINIBPhase I
MAP kinase p38 alpha inhibitor
arthritis
REGORAFENIBApproved
Discoidin domain-containing receptor 2 inhibitor
colorectal cancer
VANDETANIBApproved
Ephrin receptor inhibitor
Related Genes
EGFProtein interaction99%VEGFBProtein interaction99%PDGFCProtein interaction94%FGF3Protein interaction90%FGF5Protein interaction90%FGF6Protein interaction90%
Tissue Expression6 tissues
Heart
100%
Lung
66%
Brain
13%
Ovary
11%
Liver
11%
Bone Marrow
3%
Gene Interaction Network
Click a node to explore
TEKEGFVEGFBPDGFCFGF3FGF5FGF6
PROTEIN STRUCTURE
Preparing viewer…
PDB3BEA Β· 2.02 Γ… Β· X-ray
View on RCSB β†—
Constraintβ“˜
LOEUFβ“˜
0.37Moderately Constrained
pLIβ“˜
1.00Intolerant
Observed/Expected LoF0.28 [0.21–0.37]
RankingsWhere TEK stands among ~20K protein-coding genes
  • #1,143of 20,598
    Most Researched304 Β· top 10%
  • #534of 1,025
    FDA-Approved Drug Targets2
  • #1,151of 5,498
    Most Pathogenic Variants61 Β· top quartile
  • #1,694of 17,882
    Most Constrained (LOEUF)0.37 Β· top 10%
Genes detectedTEK
Sources retrieved25 papers
Response timeβ€”
πŸ“„ Sources
25β–Ό
1
Tumour vasculature at single-cell resolution.
PMID: 38987599
Nature Β· 2024
1.00
2
Granzyme K
PMID: 35704599
Sci Transl Med Β· 2022
0.90
3
Role of Angiopoietin-2 in Vascular Physiology and Pathophysiology.
PMID: 31108880
Cells Β· 2019
0.80
4
Targeted therapy for capillary-venous malformations.
PMID: 38880808
Signal Transduct Target Ther Β· 2024
0.70
5
Potential Involvements of Cilia-Centrosomal Genes in Primary Congenital Glaucoma.
PMID: 39337513
Int J Mol Sci Β· 2024
0.60