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VEGF binding sites were identified on the cell surface of vascular endothelial cells in

vitro and in vivo. Subsequently, it became apparent that receptors for VEGF also

occur on bone marrow-derived cells [50]. VEGF binds two highly related receptor

tyrosine kinases (RTK), VEGFR-1 and VEGFR-2. Both VEGFR-1 and VEGFR-2

have seven immunoglobulin-like (Ig-like) domains in the extracellualar domain, a

single transmembrane region, and a consensus tyrosine kinase sequence [51, 52].

VEGFR-3 (fms-like-tyrosine kinase (Flt)-4) is a member of the same family of RTKs,

but is not a receptor for VEGF, binding instead to VEGF-C and VEGF-D [53]. In

addition to these RTKs, VEGF interacts with a family of coreceptors, the neuropilins

(NRP).

VEGFRs share similar regulatory mechanisms with well-characterized receptor

tyrosine kinases, by which include receptor dimerization and activation of the tyrosine

kinase. Moreover, VEGFRs perform cellular processes that are common to many

growth factor receptors such as cell survival and proliferation. The summary of

VEGFRs shows in Figure 1, VEGF-A binds to VEGFR-1 and VEGFR-2, which are

expressed in the cell surface of most blood ECs. In contrast, PLGF and VEGFB

interact only with VEGFR-1. VEGFC and VEGFD bind VEGFR-2 and VEGFR-3,

which is largely restricted to lymphatic EC. There is much evidence that VEGFR-2 is

the major mediator of EC mitogenesis and survival, as well as angiogenesis and

micro-vascular permeability. VEGFR-1 has an established signaling role in mediating

monocyte chemotaxis. Also, in hematopoietic stem cells (HSC) or leukemic cells,

both VEGFR-1 and VEGFR-2 may mediate a chemotactic and a survival signal [43].

1.3.1 VEGFR-1

VEGFR-1 or Flt-1 (fms-like-tyrosine kinase-1) was the first discovery of VEGF

receptor [54]. The VEGFR-1 tyrosine kinase exhibits all the conserved motifs that are

required for kinase activity. The crystal structure of part of the VEGFR-1 extracellular

domain shows that the Ig domain-2 is the major ligand binding site on the receptor in

physiology and pathology. VEGFR-1 expression is unregulated by hypoxia by a

HIF-1 dependent mechanism [55].VEGFR-1 binds not only VEGF-A but also PLGF

and VEGF-B [40, 56]. The crucial role of VEGFR-1, as mentioned above, was

determined by Fong, 1995, which revealed that when disruption of VEGFR-1 gene in

mice resulted in embryonic lethality.

In addition to the full length of VEGFR-1, there is an alternatively spliced

soluble form of VEGFR-1 (sFlt-1), which has been shown to be an inhibitor of VEGF

activity [57]. Hence, not only the full length membrane bound form of VEGFR-1 but

sFlt-1 as well could perform a decoy function, which sequesters VEGF and prevent its

interaction with VEGFR-2 [58].

In some cases, VEGFR-1 is expressed by tumor cells and probably mediates a

chemotatic signal, thus potentially extending the role of this receptor in cancer growth.

For instance, Wu and collaborates indicated that VEGF-A autocrine growth activity is

acquired by certain human breast tumor cell lines defined by expression of VEGFR-1

[59].

Anchoring of the extracellular domain of VEGFR-1 to the cell membrane is

important, as 50% of the mice that lack both of the tyrosine kinase domains and the

transmembrane domain died at embryonic stage, owing to vascular malformation [60].

This study indicates that endothelial cells develop but fails to organize in vascular

channels. Excessive proliferation of angioblasts has been reported to be responsible

for such disorganization and leathality, indicating that, at least during early

development, VEGFR-1 is a negative regulator of VEGF action [61]. By contrarily,

many evidence also indicated that VEGFR-1 is a positive regulator of monocyte and

macrophage migration [62, 63].

1.3.2 VEGFR-2

At the same time VEGFR-1 was discovered, Shalaby et al. found VEGFR-2, another

receptor for VEGF, and proposed that VEGFR-2 had similar characteristics and

continuously shows today. Biochemical analyses showed that the second and third

Ig-like domains in VEGFR-2, also known as kinase domain region (KDR), is

important for the determination of ligand binding specificity for VEGF [64].

VEGFR-2 undergoes RTK dimerization and strong ligand-dependent tyrosine

phosphorylation in intact cells.

There are many studies indicating that VEGFR-2 is the major mediator of the

mitogenesis, survival, and permeability enhancing effects of VEGF-A in endothelial

cells [32]. VEGF binds to VEGFR-2 and stimulates activation of Ras in HUVECs.

Besides, Ras activation has been coupled to an angiogenic phenotype of endothelial

cells [65]. The early finding that the binding of VEGF to VEGFR-2 is enhanced by

heparin has been confirmed by recent studies, which shows that heparin amplifies

signalling by VEGF. Byzova et al. have reported that VEGFR-2 activation by VEGF

results in PI3 kinase/Akt-dependent activation of several integrins, which indicates

that VEGF enhanced cell adhesion, migration, soluble ligand binding [66]. Moreover,

VEGFR-2 activation has been shown to be required for the antiapoptotic effects of

VEGF for human umbilical vein endothelial cells (HUVECs) [29].

1.3.3 VEGFR-3 and Neuropilin

VEGFR-1 and VEGFR-2 are expressed on the surface of blood endothelial cells. By

contrast, VEGFR-3 (Flt-4) is largely restricted to lymphatic endothelial cells. In recent

studies, VEGFR-3 is important for lymphatic endothelial cell development and

function [53].

Neuropilin-1 (NRP1), as its name suggests, is a molecular that is implicated in

neuronal guidelince and had been previously shown to bind the collapsin/semaphorin

family. NRP1 is a cell surface glycoprotein that lacks intrinsic catalytic activity, a

receptor for the heparin-binding isoforms of VEGF, and seems to present VEGF165 to

VEGFR-2 in a manner that potentiates VEGFR-2 signaling [67]. This result shows

that neuropilin stabilizes the VEGF/VEGFR-2 signaling complex when expressed on

adjacent cells.

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