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Published Online
on January 30, 2003

Arteriosclerosis, Thrombosis, and Vascular Biology. 2003
Published online before print January 30, 2003, doi: 10.1161/01.ATV.0000058401.34021.D4
A more recent version of this article appeared on March 1, 2003
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Submitted on October 2, 2002
Accepted on January 9, 2003

Deficiency of ADAMTS13 Causes Thrombotic Thrombocytopenic Purpura

Han-Mou Tsai *

From the Division of Hematology, Montefiore Medical Center and Albert Einstein College of Medicine, Bronx, NY.

* To whom correspondence should be addressed. E-mail: htsai{at}montefiore.org.

Abstract--In the circulation, a plasma metalloprotease, ADAMTS13, cleaves von Willebrand factor (vWF) in a shear-dependent manner. This article reviews the role of this cleavage in regulating VWF-platelet interaction and proposes a scheme for understanding how a deficiency of ADAMTS13 results in the development of microthrombi in patients with thrombotic thrombocytopenic purpura.
Key words: von Willebrand factor • thrombotic thrombocytopenic purpura • ADAMTS13 • shear stress • von Willebrand factor cleaving protease




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