MOLECULAR AND CELLULAR BIOLOGY OF VON-WILLEBRAND-FACTOR

Von Willebrand factor (VWF), a central protein in the regulation of bl ood coagulation, serves as a major adhesive Link between platelets and the blood vessel wall and also functions as a carrier in plasma for f actor VIII. Abnormalities of vWF result in von Willebrand disease (vWD ), a common inherited human bleeding disorder Deficient von Willebrand factor function has been proposed as potentially protective against t he development of coronary vascular disease and several recent investi gational therapies are directed at the vWF-platelet interaction. This review summarizes the current state of knowledge regarding the biosynt hesis and processing of vWF and the relationship of VWF structure to f unction. Finally, recent progress in identifying specific genetic muta tions responsible for the many variants of vWD is discussed.