Imperial College London
Portrait Picture

ProfessorAnnaRandi

Faculty of Medicine, National Heart & Lung Institute

Head of Section for Vascular Science
 
 
 
//

Contact

 

a.randi Website

 
 
//

Location

 

L-block, room 533Hammersmith HospitalHammersmith Campus

//

Summary

 

Publications

Citation

BibTex format

@article{Randi:2018:10.1182/blood-2018-01-769018,
author = {Randi, AM and Smith, KE and Castaman, G},
doi = {10.1182/blood-2018-01-769018},
journal = {Blood},
pages = {132--140},
title = {von Willebrand factor regulation of blood vessel formation},
url = {http://dx.doi.org/10.1182/blood-2018-01-769018},
volume = {132},
year = {2018}
}
Download

RIS format (EndNote, RefMan)

TY  - JOUR
AB  - Several important physiological processes, from permeability to inflammation to haemostasis, take place at the vessel wall and are regulated by endothelial cells (EC). Thus, proteins that have been identified as regulators of one process are increasingly found to be involved in other vascular functions. Such is the case for Von Willebrand Factor (VWF), a large glycoprotein best known for its critical role in haemostasis. In vitro and in vivo studies have shown that lack of VWF causes enhanced vascularisation, both constitutively and following ischemia. This evidence is supported by studies on blood outgrowth endothelial cells (BOEC) from patients with lack of VWF synthesis (type 3 von Willebrand disease [VWD]). The molecular pathways are likely to involve VWF binding partners, such as integrin αvβ3, and components of Weibel Palade bodies (WPB), such as Angiopoietin-2 and Galectin-3, whose storage is regulated by VWF; these converge on the master regulator of angiogenesis and endothelial homeostasis, vascular endothelial growth factor (VEGF) signalling. Recent studies suggest that the roles of VWF may be tissue-specific. The ability of VWF to regulate angiogenesis has clinical implications for a subset of VWD patients with severe, intractable gastrointestinal bleeding due to vascular malformations. In this article, we review the evidence showing that VWF is involved in blood vessel formation, discuss the role of VWF high molecular weight multimers in regulating angiogenesis, and the value of studies on BOEC in developing a precision medicine approach to validate novel treatments for angiodysplasia in congenital VWD and acquired von Willebrand syndrome.
AU  - Randi,AM
AU  - Smith,KE
AU  - Castaman,G
DO  - 10.1182/blood-2018-01-769018
EP  - 140
PY  - 2018///
SN  - 1528-0020
SP  - 132
TI  - von Willebrand factor regulation of blood vessel formation
T2  - Blood
UR  - http://dx.doi.org/10.1182/blood-2018-01-769018
UR  - https://www.ncbi.nlm.nih.gov/pubmed/29866817
UR  - http://hdl.handle.net/10044/1/60832
VL  - 132
ER  -
Download