Regulation of von Willebrand factor-platelet interactions

Peter J. Lenting; Julie N. Pegon; Evelyn Groot; Philip G. de Groot

doi:10.1160/TH09-11-0777

Thrombosis and Haemostasis, Inhaltsverzeichnis

Thromb Haemost 2010; 104(03): 449-455
DOI: 10.1160/TH09-11-0777

Theme Issue Article

Schattauer GmbH

Regulation of von Willebrand factor-platelet interactions

Peter J. Lenting

¹INSERM U.770 and Univ. Paris-Sud, 94276, Le Kremlin-Bicêtre, France

,

Julie N. Pegon

¹INSERM U.770 and Univ. Paris-Sud, 94276, Le Kremlin-Bicêtre, France

,

Evelyn Groot

²Department of Clinical Chemistry and Haematology, University Medical Center Utrecht, Utrecht, The Netherlands

,

Philip G. de Groot

²Department of Clinical Chemistry and Haematology, University Medical Center Utrecht, Utrecht, The Netherlands

› Institutsangaben

Abstract

Summary

The formation of thrombi is a multistep process involving several components, including von Willebrand factor (VWF). VWF is an adhesive multimeric protein, which acts as a molecular bridge between the subendothelial matrix and the glycoprotein Ib/IX/V receptor complex. Furthermore, VWF promotes the expansion of the platelet plug by cross-linking platelets via binding to integrin αIIbβ3. In terms of thrombus formation, it is essential that VWF-platelet interactions occur timely, that is: it should happen not too early or too late. Given the co-existence of VWF and platelets in the circulation, this implies that there must be regulatory mechanisms that prevent premature formation of VWF-rich platelet aggregates that could occlude the vasculature. Indeed, several mechanisms have been identified at the level of VWF, which are dedicated to the prevention of excessive VWF-platelet interactions following endothelial release of VWF (which may include limited exposure to shear stress, the presence of Mg²⁺ ions, inhibition of VWF-platelet interactions by endothelial proteins, ADAMTS13-mediated proteolysis) and of circulating VWF-platelet aggregates during normal circulation (shielding of the platelet-binding A1 domain by other regions of the VWF molecule, inhibition of VWF-platelet interactions by β2-glycoprotein I). In the present review an overview of these mechanisms will be discussed.

Keywords

Von Willebrand factor - thrombosis - von Willebrand disease

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Referenzen

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