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Factor VIII and von Willebrand Factor

Journal: Thrombosis and Haemostasis
ISSN: 0340-6245
Issue: 1998: 79/3 (Mar) pp.456-690
Pages: 456-465

Factor VIII and von Willebrand Factor

André J. Vlot, Stefan J. Koppelman, Bonno N. Bouma, Jan J. Sixma
From the Department of Haematology, University Hospital Utrecht, Utrecht, The Netherlands

Summary

Factor VIII and von Willebrand factor are plasma glycoproteins whose deficiency or structural defects cause hemophilia A and von Willebrand disease, respectively (1). These diseases are the most common inherited bleeding disorders of man. Factor VIII and vWF are synthesized by different cell types and circulate in plasma as a tightly bound complex. Factor VIII is synthesized in the liver (2), and functions as a cofactor for activated factor IX in the intrinsic activation of factor X on a membrane surface (3). vWF is synthesized in endothelial cells (4, 5) and megakaryocytes (6). vWF has a dual role in hemostasis: it promotes platelet adhesion to subendothelium after vessel injury (7, 8) and it acts as a carrier protein of factor VIII (1)...