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ADAMTS13 and von Willebrand factor: a Dynamic Duo

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Title: ADAMTS13 and von Willebrand factor: a Dynamic Duo
Authors: South, K
Lane, DA
Item Type: Journal Article
Abstract: von Willebrand factor (VWF) is a key player in hemostasis, acting as a carrier for factor VIII and capturing platelets at sites of vascular damage. To capture platelets, it must undergo conformational changes, both within its A1 domain and at the macromolecular level through A2 domain unfolding. Its size and this function are regulated by the metalloproteinase ADAMTS-13. Recently, it has been shown that ADAMTS-13 undergoes a conformational change upon interaction with VWF, and that this enhances its activity towards its substrate. This review summarizes recent work on these conformational transitions, describing how they are controlled. It points to their importance in hemostasis, bleeding disorders, and the developing field of therapeutic application of ADAMTS-13 as an antithrombotic agent in obstructive microvascular thrombosis and in cardiovascular disease.
Issue Date: 2-Dec-2017
Date of Acceptance: 25-Oct-2017
URI: http://hdl.handle.net/10044/1/53061
DOI: https://dx.doi.org/10.1111/jth.13898
ISSN: 1538-7836
Publisher: Wiley
Start Page: 6
End Page: 18
Journal / Book Title: Journal of Thrombosis and Haemostasis
Volume: 16
Issue: 1
Copyright Statement: © 2017 The Authors. Journal of Thrombosis and Haemostasis published by Wiley Periodicals, Inc. on behalf of International Society on Thrombosis and Haemostasis. This is an open access article under the terms of the Creative Commons Attribution License, which permits use, distribution and reproduction in any medium, provided the original work is properly cited.
Sponsor/Funder: British Heart Foundation
British Heart Foundation
Funder's Grant Number: PG/12/55/29740
PG/14/87/3118
Keywords: Science & Technology
Life Sciences & Biomedicine
Hematology
Peripheral Vascular Disease
Cardiovascular System & Cardiology
ADAMTS-13
conformational activation
hemostasis
TTP
VWF
1102 Cardiovascular Medicine And Haematology
1103 Clinical Sciences
Cardiovascular System & Hematology
Publication Status: Published
Appears in Collections:Department of Medicine
Faculty of Medicine



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