4. Modulation of von Willebrand Factor by ADAMTS13

  1. Augusto B. Federici MD3,
  2. Christine A. Lee MA, MD, DSc (Med), FRCP, FRCPath, FRCOGad eundem4,
  3. Erik E. Berntorp MD, PhD5,
  4. David Lillicrap MD6 and
  5. Robert R. Montgomery MD7,8
  1. Jennifer Barr BS1 and
  2. David Motto MD, PhD2

Published Online: 21 MAR 2011

DOI: 10.1002/9781444329926.ch4

Von Willebrand Disease: Basic and Clinical Aspects

Von Willebrand Disease: Basic and Clinical Aspects

How to Cite

Barr, J. and Motto, D. (2011) Modulation of von Willebrand Factor by ADAMTS13, in Von Willebrand Disease: Basic and Clinical Aspects (eds A. B. Federici, C. A. Lee, E. E. Berntorp, D. Lillicrap and R. R. Montgomery), Wiley-Blackwell, Oxford, UK. doi: 10.1002/9781444329926.ch4

Editor Information

  1. 3

    Division of Hematology and Transfusion Medicine, L. Sacco University Hospital, Department of Internal Medicine, University of Milan, Milan, Italy

  2. 4

    University of London, London, UK

  3. 5

    Malmö Centre for Thrombosis and Haemostasis, Lund University, Skåne University Hospital, Malmö, Sweden

  4. 6

    Department of Pathology and Molecular Medicine, Richardson Laboratory, Queen's University, Kingston, ON, Canada

  5. 7

    Department of Pediatrics, Medical College of Wisconsin, Milwaukee, WI, USA

  6. 8

    Blood Research Institute, Blood Center of Wisconsin, Milwaukee, WI, USA

Author Information

  1. 1

    Department of Anatomy and Cell Biology, University of Iowa Carver College of Medicine, Iowa City, IA, USA

  2. 2

    Departments of Internal Medicine and Pediatrics, University of Iowa Carver College of Medicine, Iowa City, IA, USA

Publication History

  1. Published Online: 21 MAR 2011
  2. Published Print: 25 MAR 2011

ISBN Information

Print ISBN: 9781405195126

Online ISBN: 9781444329926

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Keywords:

  • ADAMTS13;
  • von Willebrand factor;
  • thrombotic thrombocytopenic purpura;
  • von Willebrand disease;
  • VWF-cleaving protease;
  • thrombotic microangiopathy

Summary

Existence of the metalloprotease now known to be ADAMTS13 was first established in 1996 through studies investigating the pathogenesis of thrombotic thrombocytopenic purpura (TTP). Since then, considerable progress has been made into unraveling the pathophysiology of this disease, including the roles played by both ADAMTS13 and the von Willebrand factor (VWF). Much of this work has contributed greatly to our understanding of how these molecules function in “normal” physiology and hemostasis as well. In this chapter we will review the discovery of ADAMTS13 and its role in TTP, recent findings regarding the regulation of ADAMTS13 activity and its interaction with VWF, and the implication of these findings to von Willebrand disease.