The Role of von Willebrand Factor in Vascular Inflammation: From Pathogenesis to Targeted Therapy

Beyond its role in hemostasis, von Willebrand factor (VWF) is an emerging mediator of vascular inflammation. Recent studies highlight the involvement of VWF and its regulator, ADAMTS13, in mechanisms that underlie vascular inflammation and immunothrombosis, like leukocyte rolling, adhesion, and extr...

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Main Authors: Felice Gragnano, Simona Sperlongano, Enrica Golia, Francesco Natale, Renatomaria Bianchi, Mario Crisci, Fabio Fimiani, Ivana Pariggiano, Vincenzo Diana, Andreina Carbone, Arturo Cesaro, Claudia Concilio, Giuseppe Limongelli, Mariagiovanna Russo, Paolo Calabrò
Format: Article
Language:English
Published: Wiley 2017-01-01
Series:Mediators of Inflammation
Online Access:http://dx.doi.org/10.1155/2017/5620314
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author Felice Gragnano
Simona Sperlongano
Enrica Golia
Francesco Natale
Renatomaria Bianchi
Mario Crisci
Fabio Fimiani
Ivana Pariggiano
Vincenzo Diana
Andreina Carbone
Arturo Cesaro
Claudia Concilio
Giuseppe Limongelli
Mariagiovanna Russo
Paolo Calabrò
author_facet Felice Gragnano
Simona Sperlongano
Enrica Golia
Francesco Natale
Renatomaria Bianchi
Mario Crisci
Fabio Fimiani
Ivana Pariggiano
Vincenzo Diana
Andreina Carbone
Arturo Cesaro
Claudia Concilio
Giuseppe Limongelli
Mariagiovanna Russo
Paolo Calabrò
author_sort Felice Gragnano
collection DOAJ
description Beyond its role in hemostasis, von Willebrand factor (VWF) is an emerging mediator of vascular inflammation. Recent studies highlight the involvement of VWF and its regulator, ADAMTS13, in mechanisms that underlie vascular inflammation and immunothrombosis, like leukocyte rolling, adhesion, and extravasation; vascular permeability; ischemia/reperfusion injury; complements activation; and NETosis. The VWF/ADAMTS13 axis is implicated in the pathogenesis of atherosclerosis, promoting plaque formation and inflammation through macrophage and neutrophil recruitment in inflamed lesions. Moreover, VWF and ADAMTS13 have been recently proposed as prognostic biomarkers in cardiovascular, metabolic, and inflammatory diseases, such as diabetes, stroke, myocardial infarction, and sepsis. All these features make VWF an attractive therapeutic target in thromboinflammation. Several lines of research have recently investigated “tailor-made” inhibitors of VWF. Results from animal models and clinical studies support the potent anti-inflammatory and antithrombotic effect of VWF antagonism, providing reassuring data on its safety profile. This review describes the role of VWF in vascular inflammation “from bench to bedside” and provides an updated overview of the drugs that can directly interfere with the VWF/ADAMTS13 axis.
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spelling doaj-art-7f7f0f03712d43b0b517a444ce155e592025-02-03T01:20:52ZengWileyMediators of Inflammation0962-93511466-18612017-01-01201710.1155/2017/56203145620314The Role of von Willebrand Factor in Vascular Inflammation: From Pathogenesis to Targeted TherapyFelice Gragnano0Simona Sperlongano1Enrica Golia2Francesco Natale3Renatomaria Bianchi4Mario Crisci5Fabio Fimiani6Ivana Pariggiano7Vincenzo Diana8Andreina Carbone9Arturo Cesaro10Claudia Concilio11Giuseppe Limongelli12Mariagiovanna Russo13Paolo Calabrò14Division of Cardiology, Department of Cardio-Thoracic and Respiratory Sciences, University of Campania “Luigi Vanvitelli” and A.O. dei Colli Monaldi Hospital, Naples, ItalyDivision of Cardiology, Department of Cardio-Thoracic and Respiratory Sciences, University of Campania “Luigi Vanvitelli” and A.O. dei Colli Monaldi Hospital, Naples, ItalyDivision of Cardiology, Department of Cardio-Thoracic and Respiratory Sciences, University of Campania “Luigi Vanvitelli” and A.O. dei Colli Monaldi Hospital, Naples, ItalyDivision of Cardiology, Department of Cardio-Thoracic and Respiratory Sciences, University of Campania “Luigi Vanvitelli” and A.O. dei Colli Monaldi Hospital, Naples, ItalyDivision of Cardiology, Department of Cardio-Thoracic and Respiratory Sciences, University of Campania “Luigi Vanvitelli” and A.O. dei Colli Monaldi Hospital, Naples, ItalyDivision of Cardiology, Department of Cardio-Thoracic and Respiratory Sciences, University of Campania “Luigi Vanvitelli” and A.O. dei Colli Monaldi Hospital, Naples, ItalyDivision of Cardiology, Department of Cardio-Thoracic and Respiratory Sciences, University of Campania “Luigi Vanvitelli” and A.O. dei Colli Monaldi Hospital, Naples, ItalyDivision of Cardiology, Department of Cardio-Thoracic and Respiratory Sciences, University of Campania “Luigi Vanvitelli” and A.O. dei Colli Monaldi Hospital, Naples, ItalyDivision of Cardiology, Department of Cardio-Thoracic and Respiratory Sciences, University of Campania “Luigi Vanvitelli” and A.O. dei Colli Monaldi Hospital, Naples, ItalyDivision of Cardiology, Department of Cardio-Thoracic and Respiratory Sciences, University of Campania “Luigi Vanvitelli” and A.O. dei Colli Monaldi Hospital, Naples, ItalyDivision of Cardiology, Department of Cardio-Thoracic and Respiratory Sciences, University of Campania “Luigi Vanvitelli” and A.O. dei Colli Monaldi Hospital, Naples, ItalyDivision of Cardiology, Department of Cardio-Thoracic and Respiratory Sciences, University of Campania “Luigi Vanvitelli” and A.O. dei Colli Monaldi Hospital, Naples, ItalyDivision of Cardiology, Department of Cardio-Thoracic and Respiratory Sciences, University of Campania “Luigi Vanvitelli” and A.O. dei Colli Monaldi Hospital, Naples, ItalyDivision of Cardiology, Department of Cardio-Thoracic and Respiratory Sciences, University of Campania “Luigi Vanvitelli” and A.O. dei Colli Monaldi Hospital, Naples, ItalyDivision of Cardiology, Department of Cardio-Thoracic and Respiratory Sciences, University of Campania “Luigi Vanvitelli” and A.O. dei Colli Monaldi Hospital, Naples, ItalyBeyond its role in hemostasis, von Willebrand factor (VWF) is an emerging mediator of vascular inflammation. Recent studies highlight the involvement of VWF and its regulator, ADAMTS13, in mechanisms that underlie vascular inflammation and immunothrombosis, like leukocyte rolling, adhesion, and extravasation; vascular permeability; ischemia/reperfusion injury; complements activation; and NETosis. The VWF/ADAMTS13 axis is implicated in the pathogenesis of atherosclerosis, promoting plaque formation and inflammation through macrophage and neutrophil recruitment in inflamed lesions. Moreover, VWF and ADAMTS13 have been recently proposed as prognostic biomarkers in cardiovascular, metabolic, and inflammatory diseases, such as diabetes, stroke, myocardial infarction, and sepsis. All these features make VWF an attractive therapeutic target in thromboinflammation. Several lines of research have recently investigated “tailor-made” inhibitors of VWF. Results from animal models and clinical studies support the potent anti-inflammatory and antithrombotic effect of VWF antagonism, providing reassuring data on its safety profile. This review describes the role of VWF in vascular inflammation “from bench to bedside” and provides an updated overview of the drugs that can directly interfere with the VWF/ADAMTS13 axis.http://dx.doi.org/10.1155/2017/5620314
spellingShingle Felice Gragnano
Simona Sperlongano
Enrica Golia
Francesco Natale
Renatomaria Bianchi
Mario Crisci
Fabio Fimiani
Ivana Pariggiano
Vincenzo Diana
Andreina Carbone
Arturo Cesaro
Claudia Concilio
Giuseppe Limongelli
Mariagiovanna Russo
Paolo Calabrò
The Role of von Willebrand Factor in Vascular Inflammation: From Pathogenesis to Targeted Therapy
Mediators of Inflammation
title The Role of von Willebrand Factor in Vascular Inflammation: From Pathogenesis to Targeted Therapy
title_full The Role of von Willebrand Factor in Vascular Inflammation: From Pathogenesis to Targeted Therapy
title_fullStr The Role of von Willebrand Factor in Vascular Inflammation: From Pathogenesis to Targeted Therapy
title_full_unstemmed The Role of von Willebrand Factor in Vascular Inflammation: From Pathogenesis to Targeted Therapy
title_short The Role of von Willebrand Factor in Vascular Inflammation: From Pathogenesis to Targeted Therapy
title_sort role of von willebrand factor in vascular inflammation from pathogenesis to targeted therapy
url http://dx.doi.org/10.1155/2017/5620314
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