Contact Person

Dr. Elinor Switzer

Managing Editor

Phone: +49 (0)711 - 2 29 87 63
Fax: +49 (0)711 - 2 29 87 65
send an Email


Regulation of von Willebrand factor-platelet interactions

Journal: Thrombosis and Haemostasis
ISSN: 0340-6245

Theme Issue: Highlights from EMVBM 2009

Issue: 2010: 104/3 (Sep) pp. 421–653
Pages: 449-455

Regulation of von Willebrand factor-platelet interactions

P. J. Lenting (1), J. N. Pegon (1), E. Groot (2), P. G. de Groot (2)

(1) INSERM U.770 and Univ. Paris-Sud, 94276, Le Kremlin-Bicêtre, France; (2) Department of Clinical Chemistry and Haematology, University Medical Center Utrecht, Utrecht, The Netherlands


thrombosis, von Willebrand factor, von Willebrand disease


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.

You may also be interested in...

Tímea Szántó 1,2, Ágota Schlammadinger 3, Stephanie Staelens 1, Simon F. De Meyer 1, Kathleen Freson 4, Inge Pareyn 1, Stephan Vauterin 1, Jolán Hársfalvi 2, Hans Deckmyn 1, Karen Vanhoorelbeke1

Thromb Haemost 2007 98 1: 178-185

Carolyn M. Millar 1, Anne F. Riddell 1, Simon A. Brown 1, Richard Starke 2, Ian Mackie 2, Derrick J. Bowen 3, P. Vincent Jenkins 1, Jan A. van Mourik4

Thromb Haemost 2008 99 5: 916-924

Alessandra Casonato, Francesca Sartorello, Elena Pontara, Lisa Gallinaro, Antonella Bertomoro, Maria Grazia Cattini, Viviana Daidone, Maryta Szukowska, Antonio Pagnan

Thromb Haemost 2007 98 6: 1182-1187