Von Willebrand disease is a congenital bleeding disorder caused by defects in the von Willebrand factor protein (VWF). VWF is thought to undergo a variety of posttranslational modifications that influence the affinity and availability for Factor VII, including cleavage of the propeptide and formation of N-terminal intersubunit disulfide bonds.VWF is a multimeric glycoprotein that is found in endothelial cells, plasma and platelets, and it is involved in the coagulation of blood at injury sites. VWF acts as a carrier protein for Factor VIII, a cofactor required for coagulation, and it promotes platelet adhesion and aggregation.
