ON THE VERSATILITY OF VON WILLEBRAND FACTOR

Von Willebrand factor (VWF) is a large multimeric protein, the function of which has been demonstrated to be pivotal to the haemostatic system. Indeed, quantitative and/or qualitative abnormalities of VWF are associated with the bleeding disorder Von Willebrand disease (VWD). Moreover, increased pla...

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Main Authors: Antoine Rauch, Peter Lenting
Format: Article
Language:English
Published: PAGEPress Publications 2013-07-01
Series:Mediterranean Journal of Hematology and Infectious Diseases
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Online Access:http://www.mjhid.org/index.php/mjhid/article/view/417
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author Antoine Rauch
Peter Lenting
author_facet Antoine Rauch
Peter Lenting
author_sort Antoine Rauch
collection DOAJ
description Von Willebrand factor (VWF) is a large multimeric protein, the function of which has been demonstrated to be pivotal to the haemostatic system. Indeed, quantitative and/or qualitative abnormalities of VWF are associated with the bleeding disorder Von Willebrand disease (VWD). Moreover, increased plasma concentrations of VWF have been linked to an increased risk for thrombotic complications. In the previous decades, many studies have contributed to our understanding of how VWF is connected to the haemostatic system, particularly with regard to structure-function relationships. Interactive sites for important ligands of VWF (such as factor VIII, collagen, glycoprotein Iba, integrin aIIbb3 and protease ADAMTS13) have been identified, and mutagenesis studies have confirmed the physiological relevance of the interactions between VWF and these ligands.  However, we have also become aware that VWF has a more versatile character than previously thought, given its potential role in various non-hemostatic processes, like intimal thickening, tumor cell apoptosis and inflammatory processes. In the presence review, a summary of our knowledge on VWF structure-function relationships is provided in the context of the "classical" haemostatic task of VWF and in perspective of pathological processes beyond haemostasis.
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spelling doaj-art-afd0a968c9d046109cfd05ac17feb2322025-01-02T06:45:40ZengPAGEPress PublicationsMediterranean Journal of Hematology and Infectious Diseases2035-30062013-07-0151e2013046e201304610.4084/mjhid.2013.046309ON THE VERSATILITY OF VON WILLEBRAND FACTORAntoine RauchPeter LentingVon Willebrand factor (VWF) is a large multimeric protein, the function of which has been demonstrated to be pivotal to the haemostatic system. Indeed, quantitative and/or qualitative abnormalities of VWF are associated with the bleeding disorder Von Willebrand disease (VWD). Moreover, increased plasma concentrations of VWF have been linked to an increased risk for thrombotic complications. In the previous decades, many studies have contributed to our understanding of how VWF is connected to the haemostatic system, particularly with regard to structure-function relationships. Interactive sites for important ligands of VWF (such as factor VIII, collagen, glycoprotein Iba, integrin aIIbb3 and protease ADAMTS13) have been identified, and mutagenesis studies have confirmed the physiological relevance of the interactions between VWF and these ligands.  However, we have also become aware that VWF has a more versatile character than previously thought, given its potential role in various non-hemostatic processes, like intimal thickening, tumor cell apoptosis and inflammatory processes. In the presence review, a summary of our knowledge on VWF structure-function relationships is provided in the context of the "classical" haemostatic task of VWF and in perspective of pathological processes beyond haemostasis.http://www.mjhid.org/index.php/mjhid/article/view/417von Willebrand FactorHemorrhageThrombosis, ADAMTS 13
spellingShingle Antoine Rauch
Peter Lenting
ON THE VERSATILITY OF VON WILLEBRAND FACTOR
Mediterranean Journal of Hematology and Infectious Diseases
von Willebrand Factor
Hemorrhage
Thrombosis, ADAMTS 13
title ON THE VERSATILITY OF VON WILLEBRAND FACTOR
title_full ON THE VERSATILITY OF VON WILLEBRAND FACTOR
title_fullStr ON THE VERSATILITY OF VON WILLEBRAND FACTOR
title_full_unstemmed ON THE VERSATILITY OF VON WILLEBRAND FACTOR
title_short ON THE VERSATILITY OF VON WILLEBRAND FACTOR
title_sort on the versatility of von willebrand factor
topic von Willebrand Factor
Hemorrhage
Thrombosis, ADAMTS 13
url http://www.mjhid.org/index.php/mjhid/article/view/417
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