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Regulated release of VWF and FVIII and the biologic implications. Pediatr Blood Cancer 2006 May 01;46(5):547-53

Date

02/14/2006

Pubmed ID

16470522

DOI

10.1002/pbc.20658

Scopus ID

2-s2.0-33645304827 (requires institutional sign-in at Scopus site)   46 Citations

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.

Author List

Haberichter SL, Shi Q, Montgomery RR

Authors

Robert R. Montgomery MD Adjunct Professor in the Pediatrics department at Medical College of Wisconsin
Qizhen Shi MD, PhD Professor in the Pediatrics department at Medical College of Wisconsin




MESH terms used to index this publication - Major topics in bold

Coagulants
Deamino Arginine Vasopressin
Factor VIII
Hemophilia A
Humans
von Willebrand Diseases
von Willebrand Factor