Regulated release of VWF and FVIII and the biologic implications.
von Willebrand factor (VWF) performs a critical function in platelet binding at the site of vascular injury and also serves as the carrier protein for coagulation factor FVIII (FVIII), protecting it from proteolytic degradation in plasma. Both proteins undergo rapid, regulated release in response to DDAVP administration in patients with mild hemophilia A or von Wille-brand disease. Here, we attempt to summarize our current understanding of the establishment of the regulated storage pool of VWF and FVIII. The data presented indicate that regulated secretion of both proteins occurs only if there is endogenous synthesis of FVIII together with VWF.
Haberichter
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SL
Department of Pediatrics, Medical College of Wisconsin and Blood Research Institute, Blood Center of Wisconsin, Milwaukee, Wisconsin 53201, USA.
Shi
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Q
Montgomery
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RR
D003029:Coagulants / Q000627:therapeutic use*; D003894:Deamino Arginine Vasopressin / Q000627:therapeutic use*; D005169:Factor VIII / Q000378:metabolism*; D006467:Hemophilia A / Q000188:drug therapy* / Q000378:metabolism; D006801:Humans; D014842:von Willebrand Diseases / Q000188:drug therapy* / Q000378:metabolism; D014841:von Willebrand Factor / Q000378:metabolism*
D003029:Coagulants; D014841:von Willebrand Factor; D005169:Factor VIII; D003894:Deamino Arginine Vasopressin