von Willebrand factor regulation of blood vessel formation
| Autor: | Koval E. Smith, Giancarlo Castaman, Anna M. Randi |
|---|---|
| Rok vydání: | 2018 |
| Předmět: |
0301 basic medicine
congenital hereditary and neonatal diseases and abnormalities Endothelium Angiogenesis Immunology Neovascularization Physiologic 030204 cardiovascular system & hematology 1102 Cardiovascular Medicine And Haematology Biochemistry Angiodysplasia 03 medical and health sciences chemistry.chemical_compound 0302 clinical medicine Von Willebrand factor hemic and lymphatic diseases von Willebrand Factor 1114 Paediatrics And Reproductive Medicine Von Willebrand disease medicine Animals Humans Neovascularization Pathologic biology business.industry Review Series Endothelial Cells 1103 Clinical Sciences Cell Biology Hematology medicine.disease Vascular endothelial growth factor von Willebrand Diseases Vascular endothelial growth factor A 030104 developmental biology medicine.anatomical_structure Gene Expression Regulation chemistry Hemostasis cardiovascular system Cancer research biology.protein Blood Vessels Endothelium Vascular business Biomarkers Signal Transduction circulatory and respiratory physiology Blood vessel |
| Zdroj: | Blood. 132:132-140 |
| ISSN: | 1528-0020 0006-4971 |
| DOI: | 10.1182/blood-2018-01-769018 |
| Popis: | Several important physiological processes, from permeability to inflammation to hemostasis, take place at the vessel wall and are regulated by endothelial cells (ECs). 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 hemostasis. In vitro and in vivo studies have shown that lack of VWF causes enhanced vascularization, both constitutively and following ischemia. This evidence is supported by studies on blood outgrowth EC (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, 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 signaling. 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 resulting from 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 review 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. |
| Databáze: | OpenAIRE |
| Externí odkaz: |
|