Vascular endothelial growth factor receptor 3
Also known as: FLT4, VEGFR3
Function
Tyrosine-protein kinase that acts as a cell-surface receptor for VEGFC and VEGFD, and plays an essential role in adult lymphangiogenesis and in the development of the vascular network and the cardiovascular system during embryonic development. Promotes proliferation, survival and migration of endothelial cells, and regulates angiogenic sprouting. Signaling by activated FLT4 leads to enhanced production of VEGFC, and to a lesser degree VEGFA, thereby creating a positive feedback loop that enhances FLT4 signaling. Modulates KDR signaling by forming heterodimers. The secreted isoform 3 may function as a decoy receptor for VEGFC and/or VEGFD and play an important role as a negative regulator of VEGFC-mediated lymphangiogenesis and angiogenesis. Binding of vascular growth factors to isoform 1 or isoform 2 leads to the activation of several signaling cascades; isoform 2 seems to be less efficient in signal transduction, because it has a truncated C-terminus and therefore lacks several phosphorylation sites. Mediates activation of the MAPK1/ERK2, MAPK3/ERK1 signaling pathway, of MAPK8 and the JUN signaling pathway, and of the AKT1 signaling pathway. Phosphorylates SHC1. Mediates phosphorylation of PIK3R1, the regulatory subunit of phosphatidylinositol 3-kinase. Promotes phosphorylation of MAPK8 at 'Thr-183' and 'Tyr-185', and of AKT1 at 'Ser-473'.
Classification
- Family (Pfam)
- PF07679 I-set, PF13927 Ig_3, PF22971 Ig_VEGFR-1-like_5th, PF07714 PK_Tyr_Ser-Thr, PF21339 VEGFR-1-like_Ig-like, PF17988 VEGFR-2_TMD, PF22854 VEGFR1-3_N_Ig-like
- InterPro
- Ig-like_dom, Ig-like_dom_sf, Ig-like_fold, Ig_I-set, Ig_sub, Ig_sub2, Kinase-like_dom_sf, Prot_kinase_dom, Protein_kinase_ATP_BS, RTK, Ser-Thr/Tyr_kinase_cat_dom, Tyr_kinase_AS, Tyr_kinase_cat_dom, Tyr_kinase_rcpt_3_CS, VEGFR-2_TMD, VEGFR1-3_5th, VEGFR1-3_N_Ig-like
- Functional cluster
- Immunoglobulin-Domain Cell Adhesion Proteins
Experimental structures · PDB · 2
A predicted model is available from AlphaFold.
Gene Ontology · 34
- GO:0005829 cytosol
- GO:0005576 extracellular region
- GO:0005634 nucleus
- GO:0005886 plasma membrane
- GO:0043235 receptor complex
- GO:0005524 ATP binding
- GO:0019838 growth factor binding
- GO:0042803 protein homodimerization activity
- GO:0019903 protein phosphatase binding
- GO:0004714 transmembrane receptor protein tyrosine kinase activity
- GO:0005021 vascular endothelial growth factor receptor activity
- GO:0048514 blood vessel morphogenesis
- GO:0016477 cell migration
- GO:0007169 cell surface receptor protein tyrosine kinase signaling pathway
- GO:0035924 cellular response to vascular endothelial growth factor stimulus
- GO:0001945 lymph vessel development
- GO:0001946 lymphangiogenesis
- GO:0043066 negative regulation of apoptotic process
- GO:0018108 peptidyl-tyrosine phosphorylation
- GO:0030335 positive regulation of cell migration
- GO:0008284 positive regulation of cell population proliferation
- GO:0010595 positive regulation of endothelial cell migration
- GO:0001938 positive regulation of endothelial cell proliferation
- GO:0070374 positive regulation of ERK1 and ERK2 cascade
- GO:0046330 positive regulation of JNK cascade
- GO:0043410 positive regulation of MAPK cascade
- GO:0001934 positive regulation of protein phosphorylation
- GO:0010575 positive regulation of vascular endothelial growth factor production
- GO:0046777 protein autophosphorylation
- GO:0060312 regulation of blood vessel remodeling
- GO:0002040 sprouting angiogenesis
- GO:0048010 vascular endothelial growth factor receptor signaling pathway
- GO:0038084 vascular endothelial growth factor signaling pathway
- GO:0001944 vasculature development
Disease associations
- lymphatic malformation 1 MONDO:0007919
- capillary infantile hemangioma MONDO:0011191
- congenital heart defects, multiple types, 7 MONDO:0032913
Drugs targeting this protein · 43
- VATALANIB inhibitor
- SORAFENIB TOSYLATE inhibitor
- PAZOPANIB HYDROCHLORIDE inhibitor
- FORETINIB inhibitor
- FAMITINIB inhibitor
- TIVOZANIB inhibitor
- AXITINIB inhibitor
- CEP-5214 inhibitor
- SUNITINIB MALATE inhibitor
- OSI-930 inhibitor
- SU-014813 inhibitor
- REGORAFENIB inhibitor
- ILORASERTIB inhibitor
- RG-1530 inhibitor
- CEP-11981 inhibitor
- TELATINIB inhibitor
- LENVATINIB MESYLATE inhibitor
- TIVOZANIB HYDROCHLORIDE inhibitor
- TAK-593 inhibitor
- LUCITANIB inhibitor
- LINIFANIB inhibitor
- VANDETANIB inhibitor
- BRIVANIB ALANINATE inhibitor
- SEMAXANIB inhibitor
- NINTEDANIB ESYLATE inhibitor
- JNJ-26483327 inhibitor
- L-21649 inhibitor
- CEP-7055 inhibitor
- AG-13958 inhibitor
- BMS-690514 inhibitor
- BRIVANIB inhibitor
- GLESATINIB inhibitor
- KRN-633 inhibitor
- SURUFATINIB inhibitor
- CATEQUENTINIB inhibitor
- FRUQUINTINIB inhibitor
- CEDIRANIB inhibitor
- IBCASERTIB inhibitor
- DOVITINIB inhibitor
- XL-999 inhibitor
- 4SC-203 inhibitor
- SUNITINIB inhibitor
- MOTESANIB inhibitor
Related proteins · sequence + function similarity
- Vascular endothelial growth factor receptor 3 0.98
- Vascular endothelial growth factor receptor 3 0.98
- Vascular endothelial growth factor receptor 3 0.87
- Vascular endothelial growth factor receptor 1 0.87
- Vascular endothelial growth factor receptor 1 0.87
- Vascular endothelial growth factor receptor 3 0.85
- Vascular endothelial growth factor receptor 2 0.84
- Macrophage colony-stimulating factor 1 receptor 0.83
- Tyrosine-protein kinase transforming protein fms 0.83
- Vascular endothelial growth factor receptor 2 0.83
- Vascular endothelial growth factor receptor 1 0.83
- Macrophage colony-stimulating factor 1 receptor 0.82
Co-cited proteins · studied together in the literature
- Vascular endothelial growth factor receptor 3 2 shared papers
- Vascular endothelial growth factor C 2 shared papers
- Vascular endothelial growth factor receptor 2 7 shared papers
- Vascular endothelial growth factor D 1 shared papers
- SHC-transforming protein 1 1 shared papers
- Vascular endothelial growth factor receptor 1 4 shared papers
- Tyrosine-protein phosphatase non-receptor type 14 1 shared papers
- Tyrosine-protein phosphatase non-receptor type 14 1 shared papers
- Embryonic growth/differentiation factor 1 1 shared papers
- Dynein assembly factor with WD repeat domains 1 1 shared papers
- Adapter molecule crk 1 shared papers
- Vascular endothelial growth factor receptor 2 1 shared papers
Literature · 45 cited papers
- Haploinsufficiency of vascular endothelial growth factor related signaling genes is associated with tetralogy of Fallot. Genet. Med. · 2019
- Contribution of rare inherited and de novo variants in 2,871 congenital heart disease probands. Nat. Genet. · 2017
- A novel missense mutation in FLT4 causes autosomal recessive hereditary lymphedema. Lymphat. Res. Biol. · 2015
- Structural and mechanistic insights into VEGF receptor 3 ligand binding and activation. Proc. Natl. Acad. Sci. U.S.A. · 2013
- Signal transduction by vascular endothelial growth factor receptors. Biochem. J. · 2011
- Combined blockade of VEGFR-2 and VEGFR-3 inhibits inflammatory lymphangiogenesis in early and middle stages. Invest. Ophthalmol. Vis. Sci. · 2011
- Lymphangiogenesis and cancer metastasis. Front. Biosci. · 2011
- Protein tyrosine phosphatase PTPN14 is a regulator of lymphatic function and choanal development in humans. Am. J. Hum. Genet. · 2010
- Ephrin-B2 controls VEGF-induced angiogenesis and lymphangiogenesis. Nature · 2010
- Endothelial cell adhesion to the extracellular matrix induces c-Src-dependent VEGFR-3 phosphorylation without the activation of the receptor intrinsic kinase activity. Circ. Res. · 2010
- VEGF receptor 2/-3 heterodimers detected in situ by proximity ligation on angiogenic sprouts. EMBO J. · 2010
- Autocrine loop between vascular endothelial growth factor (VEGF)-C and VEGF receptor-3 positively regulates tumor-associated lymphangiogenesis in oral squamoid cancer cells. Am. J. Pathol. · 2009
- … and 33 more in the literature graph