Vascular endothelial growth factor receptor 3 (VEGFR3) (IPR009137)

Short name: VEGFR3_rcpt

Overlapping homologous superfamilies


Family relationships



Vascular endothelial growth factor (VEGF) is a potent and specific endothelial cell mitogen that regulates blood and lymphatic vessel development and homeostasis [PMID: 2478587, PMID: 7526212]. EGFs are predominantly produced by endothelial, hematopoietic, and stromal cells in response to hypoxia and upon stimulation by growth factors such as transforming growth factor beta (TGFbeta), interleukins, or platelet-derived growth factors [PMID: 19290920]. VEGFs specifically interact with one or several receptor tyrosine kinases, VEGF receptors, and with distinct co-receptors such as neuropilins or heparan sulphate glycosaminoglycans. The VEGF receptor family consists of three members, VEGFR1 (FLT1), VEGFR2 (KDR/FLK1) and VEGFR3 (FLT4) [PMID: 7538139]. Among these receptors, VEGFR1 binds strongest to VEGF, VEGF2 binds more weakly, and VEGFR3 shows essentially no binding, although it does bind to other members of the VEGF family. VEGF receptors have a characteristic structure, with 7 Ig-like domains in the extracellular domain and a cytoplasmic tyrosine kinase domain with a long kinase insert region. VEGF receptors are activated upon ligand-mediated dimerisation.

This entry represents the VEGFR3 proteins. In humans, an A to G transition corresponding to a histidine to arginine substitution in the catalytic loop of VEGFR3 has been linked to congenital hereditary lymphoedema. In vitro expression studies show that this amino acid substitution causes loss of VEGFR3 tyrosine kinase activity. Thus, defective VEGFR3 signalling is responsible for 5q34-q35-linked congenital hereditary lymphedema.

GO terms

Biological Process

GO:0006468 protein phosphorylation
GO:0048010 vascular endothelial growth factor receptor signaling pathway

Molecular Function

GO:0005524 ATP binding
GO:0005021 vascular endothelial growth factor-activated receptor activity

Cellular Component

GO:0005887 integral component of plasma membrane

Contributing signatures

Signatures from InterPro member databases are used to construct an entry.