The secretion of von Willebrand factor from endothelial cells; an increasingly complicated story
Article first published online: 30 JUN 2013
© 2013 International Society on Thrombosis and Haemostasis
Journal of Thrombosis and Haemostasis
Special Issue: State of the Art 2013
Volume 11, Issue Supplement s1, pages 192–201, June 2013
How to Cite
The secretion of von Willebrand factor from endothelial cells; an increasingly complicated story. J Thromb Haemost 2013; 11 (Suppl. 1): 192–201., .
- Issue published online: 30 JUN 2013
- Article first published online: 30 JUN 2013
- endothelial cells;
- Rab GTPase;
- von Willebrand factor;
- Weibel–Palade bodies
von Willebrand factor (VWF) plays key roles in both primary and secondary hemostasis by capturing platelets and chaperoning clotting factor VIII, respectively. It is stored within the Weibel–Palade bodies (WPBs) of endothelial cells as a highly prothrombotic protein, and its release is thus necessarily under tight control. Regulating the secretion of VWF involves multiple layers of cellular machinery that act together at different stages, leading to the exocytic fusion of WPBs with the plasma membrane and the consequent release of VWF. This review aims to provide a snapshot of the current understanding of those components, in particular the members of the Rab family, acting in the increasingly complex story of VWF secretion.