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Regulated release of VWF and FVIII and the biologic implications
Author(s) -
Haberichter S.L.,
Shi Q.,
Montgomery R.R.
Publication year - 2006
Publication title -
pediatric blood and cancer
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.116
H-Index - 105
eISSN - 1545-5017
pISSN - 1545-5009
DOI - 10.1002/pbc.20658
Subject(s) - von willebrand factor , coagulation , medicine , secretion , endogeny , von willebrand disease , platelet , immunology , function (biology) , microbiology and biotechnology , cancer research , biology
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. © 2006 Wiley‐Liss, Inc.