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Semin Thromb Hemost 2014; 40(01): 028-033
DOI: 10.1055/s-0033-1363156
Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.

The Potential Therapeutic Benefit of Targeting ADAMTS13 Activity

Elise S. Eerenberg
1   Department of Vascular Medicine, Academic Medical Center, Amsterdam, The Netherlands
,
Marcel Levi
1   Department of Vascular Medicine, Academic Medical Center, Amsterdam, The Netherlands
› Author Affiliations
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Publication History

Publication Date:
13 December 2013 (online)

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Abstract

Platelet–vessel wall interaction is mediated by von Willebrand factor (VWF), which thereby plays a major role in physiological hemostasis and thrombotic disease. VWF is released as ultralarge multimers from endothelial cells, whereupon it is cleaved by ADAMTS13 (a disintegrin and metalloproteinase with thrombospondin type I repeats-13). The prohemostatic properties of VWF are dependent of its multimeric size; hence, ADAMTS13 activity is an important determinant in platelet–vessel wall interaction. Deficiency of ADAMTS13 in its most classical form in thrombotic thrombocytopenic purpura can lead to severe thrombotic microangiopathy. However, there is a growing variety of diseases in which ADAMTS13 levels have been found to be decreased and in which reduced cleavage of VWF may play a role. Hence, targeting of VWF cleavage by pharmacological modulation of ADAMTS13 levels is an interesting approach in some of these conditions. This review discusses the available evidence for a role of ADAMTS13 in various disease states and the potential therapeutic benefit of restoration of ADAMTS13 levels.

Keywords

ADAMTS13 - von Willebrand factor - thrombotic microangiopathy
 

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