Expression of a structurally constrained von Willebrand factor variant triggers acute thrombotic thrombocytopenic purpura in mice-Reference-Cited by-同舟云学术

Expression of a structurally constrained von Willebrand factor variant triggers acute thrombotic thrombocytopenic purpura in mice

Published:2014-05-22 Issue:21 Volume:123 Page:3344-3353
ISSN:0006-4971
Container-title:Blood
language:en
Short-container-title:

Author:

Morioka Yoko¹,Casari Caterina²,Wohner Nikolett²,Cho Sungyun³,Kurata Sachiko¹,Kitano Ayumi¹,Christophe Olivier D.²,Lenting Peter J.²,Li Renhao³,Denis Cécile V.²,Prévost Nicolas¹

Affiliation:

1. Kyoto University, Career-Path Unit for Young Life Scientists, Kyoto, Japan;

2. Inserm Unité 770, Université Paris-Sud, Unité Mixte de Recherche-S 770, Le Kremlin-Bicêtre, France; and

3. Aflac Cancer and Blood Disorders Center, Department of Pediatrics, Emory University School of Medicine, Atlanta, GA

Abstract

Key Points Introduction of a disulfide bond within the A2 domain renders VWF highly thrombogenic and resistant to proteolysis. Expression of mVWF/p.S1494C-p.A1534C in mice triggers an acute onset of thrombotic thrombocytopenic purpura.

Publisher

American Society of Hematology

Subject

Cell Biology,Hematology,Immunology,Biochemistry

Link

http://ashpublications.org/blood/article-pdf/123/21/3344/1377461/3344.pdf

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