[HTML][HTML] Update on the pathophysiology and classification of von Willebrand disease: a report of the Subcommittee on von Willebrand Factor

JE Sadler, U Budde, JCJ Eikenboom… - Journal of thrombosis …, 2006 - Elsevier
Summary von Willebrand disease (VWD) is a bleeding disorder caused by inherited defects
in the concentration, structure, or function of von Willebrand factor (VWF). VWD is classified …

Function of von Willebrand factor in haemostasis and thrombosis

AJ Reininger - Haemophilia, 2008 - Wiley Online Library
The physiological protection against bleeding is secured by platelet adhesion to the site of
injury and sealing of the defect. The first step involves the arrest of platelets that have …

von Willebrand disease (VWD): evidence‐based diagnosis and management guidelines, the National Heart, Lung, and Blood Institute (NHLBI) Expert Panel report …

WL Nichols, MB Hultin, AH James… - …, 2008 - Wiley Online Library
von Willebrand disease (VWD) is a commonly encountered inherited bleeding disorder
affecting both males and females, causing mucous membrane and skin bleeding symptoms …

Mechanoenzymatic cleavage of the ultralarge vascular protein von Willebrand factor

X Zhang, K Halvorsen, CZ Zhang, WP Wong… - Science, 2009 - science.org
Von Willebrand factor (VWF) is secreted as ultralarge multimers that are cleaved in the A2
domain by the metalloprotease ADAMTS13 to give smaller multimers. Cleaved VWF is …

FRETS‐VWF73, a first fluorogenic substrate for ADAMTS13 assay

K Kokame, Y Nobe, Y Kokubo… - British journal of …, 2005 - Wiley Online Library
A plasma metalloprotease, ADAMTS13, cleaves von Willebrand factor (VWF) multimers and
downregulates their activity in platelet aggregation. Functional ADAMTS13 deficiency leads …

The genetic basis of von Willebrand disease

AC Goodeve - Blood reviews, 2010 - Elsevier
The common autosomally inherited mucocutaneous bleeding disorder, von Willebrand
disease (VWD) results from quantitative or qualitative defects in plasma von Willebrand …

Structural specializations of A2, a force-sensing domain in the ultralarge vascular protein von Willebrand factor

Q Zhang, YF Zhou, CZ Zhang… - Proceedings of the …, 2009 - National Acad Sciences
The lengths of von Willebrand factor (VWF) concatamers correlate with hemostatic potency.
After secretion in plasma, length is regulated by hydrodynamic shear force-dependent …

Unwinding the von Willebrand factor strings puzzle

K De Ceunynck, SF De Meyer… - Blood, The Journal of …, 2013 - ashpublications.org
Abstract von Willebrand factor (VWF) is amongst others synthesized by endothelial cells and
stored as ultra-large (UL) VWF multimers in Weibel-Palade bodies. Although UL-VWF is …

[HTML][HTML] von Willebrand factor: two sides of a coin

JE Sadler - Journal of thrombosis and haemostasis, 2005 - Elsevier
Everyone experiences minor bleeding and clotting, and many illnesses feature extremes of
hemorrhage or thrombosis. Recent advances have illuminated the ways in which von …

Platelet-VWF complexes are preferred substrates of ADAMTS13 under fluid shear stress

K Shim, PJ Anderson, EA Tuley… - Blood, The Journal …, 2008 - ashpublications.org
Endothelial cells secrete prothrombotic ultralarge von Willebrand factor (VWF) multimers,
and the metalloprotease ADAMTS13 cleaves them into smaller, less dangerous multimers …