Journal
PEDIATRIC BLOOD & CANCER
Volume 46, Issue 5, Pages 547-553Publisher
WILEY
DOI: 10.1002/pbc.20658
Keywords
von Willebrand factor; von Willebrand disease; factor VIII hemophilia A; protein trafficking; regulated release
Categories
Ask authors/readers for more resources
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 Willebrand 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.
Authors
I am an author on this paper
Click your name to claim this paper and add it to your profile.
Reviews
Recommended
No Data Available