In response to blood vessel damage, von Willebrand factor (vWF) binds to the exposed extra cellular matrix, recruits platelets to the site of injury, and activates platelets, which promotes thrombis formation. Patients with von Willebrand disease type 2B (vWD-type 2B) produce a vWF protein that has a high binding affinity for platelets; however, these patients exhibit a bleeding tendency that is thought to be due to loss of vWF multimers. In this issue of the Journal of Clinical Investigation , Marijke Bryckaert and colleagues at the Hôpital Kremlin Bicêtre determined that the bleeding phenotype associated with vWD-type 2B might be due to platelet dysfunction. Evaluation of platelets treated with vWD-type 2B-associated vWF revealed the mutatnt vWF was able to bind platelets, but was unable to activate them, thereby inhibiting thrombus formation. In an accompanying commentary, Jerry Ware of the University of Arkansas discusses the implications of this study for treatment of vWD-type 2B.
TITLE: von Willebrand factor mutation promotes thrombocytopathy by inhibiting integrin αIIbβ3
AUTHOR CONTACT: Marijke Bryckaert
Inserm U770, Hôpital Kremlin Bicêtre, Le Kremlin Bicêtre, UNK, FRA
Phone: 33 149595642; Fax: 33 146719472; E-mail: marijke.bryckaert@inserm.fr
View this article at: http://www.jci.org/articles/view/69458?key=3f538e18596a90b7d9e6
ACCOMPANYING COMMENTARY
TITLE: Thrombocytopathy and type 2B von Willebrand disease
AUTHOR CONTACT: Jerry Ware
University of Arkansas for Medical Sciences, Little Rock, USA
Phone: 501-526-6096; Fax: E-mail: jware@uams.edu
View this article at: http://www.jci.org/articles/view/73169?key=55eaefd5aca6c9c0b9af
Journal of Clinical Investigation