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Pediatr Blood Cancer. 2006 May 1;46(5):547-53.

Regulated release of VWF and FVIII and the biologic implications.

Author information

1
Department of Pediatrics, Medical College of Wisconsin and Blood Research Institute, Blood Center of Wisconsin, Milwaukee, Wisconsin 53201, USA.

Abstract

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.

PMID:
16470522
DOI:
10.1002/pbc.20658
[Indexed for MEDLINE]

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