Von Willebrand Disease

Excerpt

Von Willebrand factor is a glycoprotein that plays a part in hemostasis. It is synthesized in endothelial cells and megakaryocytes. After transcription and translation, pro-vWF is covalently linked to form dimers in the endoplasmic reticulum, and subsequently, large dimers form in the Golgi complex and secretory granules. The pro-von Willebrand factor propeptide then undergoes cleavage, and then both the propeptide and mature von Willebrand factor are secreted into the vessel lumen. It functions as a carrier for factor VIII to maintain its levels and help in platelet adhesion and binding to endothelial components after a vascular injury. Any qualitative or quantitative deficiency of pro-von Willebrand factor will lead to an increased bleeding tendency, and this syndrome is called Von Willebrand disease.