Von Willebrand factor

Last revised by Jeremy Jones on 6 Oct 2022

Von Willebrand factor (vWF) is a large hemostatic glycoprotein which serves several roles in platelet aggregation and the clotting cascade.

vWF is synthesized by vascular endothelial cells and megakaryocytes. A series of complex post-translational modifications result in the creation of physiologically active large-molecular-weight vWF multimers (LMWMs) and ultra large-molecular-weight multimers (ULMWMs) 1. It is encoded by the large VWF gene on the short arm of chromosome 12 3

The active forms of vWF are released by injured endothelium, which promotes hemostasis by three mechanisms 2,3:

  • platelet adhesion: vWF binds to exposed collagen, which allows binding of GpIb resulting in platelet adhesion

  • platelet aggregation: vWF on adhered activated platelets irreversibly binds to GpIIb/IIIa to form the initial platelet plug

  • coagulation: vWF binds and stabilizes factor VIII in the clotting cascade, prolonging its half-life

Ongoing research is identifying further interactions of vWF in inflammation, hemorrhagic shock and traumatic brain injury 3.

Von Willebrand disease is a bleeding diathesis due to a deficiency in vWF. It is the most common inherited disorder of hemostasis.

It is named after Erik Adolf von Willebrand (1870-1949), Finnish physician and medical professor, who first described von Williebrand disease in 1926 2.

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