Article
Expression of a structurally constrained von Willebrand factor variant triggers acute thrombotic thrombocytopenic purpura in mice.
Blood - 22 May 2014
Morioka Yoko, Casari Caterina, Wohner Nikolett, Cho Sungyun, Kurata Sachiko, Kitano Ayumi, Christophe Olivier D, Lenting Peter J, Li Renhao, Denis Cécile V, Prévost Nicolas
Abstract excerpt
Thrombotic thrombocytopenic purpura (TTP) is a life-threatening disease that presents with thrombocytopenia, disseminated thrombosis, hemolytic anemia, and organ dysfunction. The etiology of TTP has revealed that patients share a deficiency in plasma protease a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13 (ADAMTS13), the enzyme responsible for cleaving ultra-large von Willebrand...
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