Von Willebrand factor, platelets and endothelial cell interactions

Abstract

To cite this article: Ruggeri ZM. Von Willebrand factor, platelets and endothelial cell interactions. J Thromb Haemost 2003; 1: 1335-42.Summary. The adhesive protein von Willebrand factor (VWF) contributes to platelet function by mediating the initiation and progression of thrombus formation at sites of vascular injury. In recent years there has been considerable progress in explaining the biological properties of VWF, including the structural and functional characteristics of specific domains. The mechanism of interaction between the VWF A1 domain and glycoprotein Iba has been elucidated in detail, bringing us closer to understanding how this adhesive bond can oppose the fluid dynamic effects of rapidly flowing blood contributing to platelet adhesion and activation. Moreover, novel findings have been obtained on the link between regulation of VWF multimer size and microvascular thrombosis. This progress in basic research has provided critical information to define with greater precision the role of VWF in vascular biology and pathology, including its possible involvement in the onset of atherosclerosis and its acute thrombotic complications.

The simulated microgravity enhances multipotential differentiation capacity of bone marrow mesenchymal stem cells

“…Vascular endothelial cells synthesize and secrete VWF. VWF is only expressed in normal endothelial cells (Ruggeri 2003). Thus, they are regarded as endothelial-specific markers.…”

von Willebrand factor and factor VIII are independently required to form stable occlusive thrombi in injured veins

“…VWF has 2 main receptors, GPIb␣ in the GPIb-IX-V complex and the integrin ␣IIb␤3. 8 Pathological thrombosis can occur in arteries and veins. Arterial thrombosis is often linked to inappropriate platelet activation and can result in myocardial infarction and ischemic stroke, while venous thrombosis (eg, deep vein thrombosis) is commonly linked to high procoagulant activity, which produces fibrin-rich thrombi.…”

Relevant role of von Willebrand factor in neutrophil recruitment in a mouse sepsis model involving cecal ligation and puncture

“…Thus, VWF likely contributes to adequate leukocyte recruitment at inflammatory loci in response to severe microbial infection, possibly by supporting leukocyte extravasation from the microcirculation where blood flow creates high shear stress. 1 Neutrophils that accumulate at the site of inflammation could encapsulate microbes and prevent their systemic dissemination. In addition to neutrophil recruitment, the beneficial effects of VWF in sepsis may be explained by the recently proposed concept of "immunothrombosis".…”

“…Shogo Kasuda, 1 Hideto Matsui,2 Shiro Ono, 2 Yasunori Matsunari, 2 Kenji Nishio, 3 Midori Shima, 4 Katsuhiko Hatake, 1 …”

“…1,2 From the standpoint of the human defense system, VWF could, therefore, be a key protein serving as a functional link between inflammation and thrombosis. 2 Indeed, recent mouse model studies demonstrated that proper functional regulation of VWF-dependent inflammatory responses by ADAMTS13 considerably suppressed disease progression in patients with brain stroke 3,4 or myocardial infarction.…”