Von Willebrand Factor, ADAMTS-13, and Thrombotic Thrombocytopenic Purpura

Zhou Zhou; Trung C Nguyen; Prasenjit Guchhait; Jing-fei Dong

doi:10.1055/s-0030-1248726

Seminars in Thrombosis and Hemostasis, Table of Contents

Semin Thromb Hemost 2010; 36(1): 071-081
DOI: 10.1055/s-0030-1248726

Von Willebrand Factor, ADAMTS-13, and Thrombotic Thrombocytopenic Purpura

Zhou Zhou¹ , Trung C. Nguyen¹ ^, ² , Prasenjit Guchhait¹ , Jing-fei Dong¹

¹Department of Medicine, Thrombosis Division, Section of Cardiovascular Science Research, Baylor College of Medicine, Houston, Texas
²Department of Pediatrics, Section of Critical Care, Baylor College of Medicine, Houston, Texas

Abstract

ABSTRACT

For a disease with <80 years of history, clinical and basic research into thrombotic thrombocytopenic purpura (TTP) has been significantly accelerated since the identification of unusually large von Willebrand factor (VWF) multimers and deficiency of ADAMTS-13 (A Disintegrin And Metalloproteinase with Thrombospondin-1-like domains) as the potential cause. The VWF-cleaving metalloprotease ADAMTS-13 has since been extensively characterized and its biological action tested in vitro and in vivo. There have also been considerable efforts to understand the interaction between ADAMTS-13 and its substrate VWF, as well as its biological regulation. This review focuses on recent advances in our understanding of the biology of VWF cleavage by ADAMTS-13 and how this newly gained knowledge will eventually help the clinical management of patients with TTP. This review also discusses the potential for ADAMTS-13 as a therapeutic drug for thrombotic conditions other than TTP.

KEYWORDS

von Willebrand factor - ADAMTS-13 - fluid shear stress - thrombotic thrombocytopenic purpura - TTP

Full Text

References

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Zhou ZhouM.D. Ph.D.

Thrombosis Division, Section of Cardiovascular Research, Department of Medicine

Baylor College of Medicine, Houston, TX

Email: zz144319@bcm.tmc.edu