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[Biosynthesis in the vascular endothelial cells, molecular structure and function of von Willebrand factor]
1First Department of Internal Medicine, Niigata University School of Medicine.
von Willebrand factor (vWf) is synthesized by vascular endothelial cells and megakaryocytes, and is present in plasma, platelets and subendothelium as a large multimeric glycoprotein that has a dual role in hemostasis. vWf mediates the adhesion of platelets at the site of vascular injury by linking to specific platelet membrane receptors (glycoprotein [GP] Ib-IX complex) and to constituents of subendothelial connective tissue. vWf also functions as a carrier protein for factor VIII; this interaction is necessary for normal factor VIII survival in the circulating plasma. Each vWf subunit has binding sites for collagen, heparin, GP Ib, GPIIb/IIIa and factor VIII. Deficiency of vWf results in defective platelet adhesion and a secondary deficiency of factor VIII, both causing abnormal bleeding. In addition, vWf plays an important role in thrombogenesis and the development of atherosclerosis.
von Willebrand factor (vWf) is synthesized by vascular endothelial cells and megakaryocytes, and is present in plasma, platelets and subendothelium as a large multimeric glycoprotein that has a dual role in hemostasis. vWf mediates the adhesion of platelets at the site of vascular injury by linking to specific platelet membrane receptors (glycoprotein [GP] Ib-IX complex) and to constituents of subendothelial connective tissue. vWf also functions as a carrier protein for factor VIII; this interaction is necessary for normal factor VIII survival in the circulating plasma. Each vWf subunit has binding sites for collagen, heparin, GP Ib, GPIIb/IIIa and factor VIII. Deficiency of vWf results in defective platelet adhesion and a secondary deficiency of factor VIII, both causing abnormal bleeding. In addition, vWf plays an important role in thrombogenesis and the development of atherosclerosis.