5. Animal Models in von Willebrand Disease

  1. Augusto B. Federici MD2,
  2. Christine A. Lee MA, MD, DSc (Med), FRCP, FRCPath, FRCOGad eundem3,
  3. Erik E. Berntorp MD, PhD4,
  4. David Lillicrap MD5 and
  5. Robert R. Montgomery MD6,7
  1. Cécile V. Denis PhD,
  2. Olivier D. Christophe PhD and
  3. Peter J. Lenting PhD

Published Online: 21 MAR 2011

DOI: 10.1002/9781444329926.ch5

Von Willebrand Disease: Basic and Clinical Aspects

Von Willebrand Disease: Basic and Clinical Aspects

How to Cite

Denis, C. V., Christophe, O. D. and Lenting, P. J. (2011) Animal Models in von Willebrand Disease, 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.ch5

Editor Information

  1. 2

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

  2. 3

    University of London, London, UK

  3. 4

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

  4. 5

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

  5. 6

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

  6. 7

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

Author Information

  1. INSERM Unit 770, Université Paris-sud, Le Kremlin-Bicêtre, France

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:

  • von Willebrand disease;
  • von Willebrand factor;
  • animal models;
  • hemostasis;
  • thrombosis;
  • modifier gene

Summary

Similar to humans with von Willebrand disease (VWD), animals affected with this disorder display a bleeding phenotype. The different subtypes of the disease (1, 2, and 3) are represented in animals, making these models invaluable tools for exploring the characteristics of VWD and for testing new treatments. This chapter will provide an update of these various models, both spontaneous and genetically engineered. Their contribution in uncovering unsuspected functions of the von Willebrand factor (VWF) in diverse pathologies besides VWD and in elucidating some aspects of VWF biology not easily addressed through in vitro approaches will also be presented.