Article
von Willebrand factor cleaving protease and ADAMTS13 mutations in childhood TTP.
Blood - 1 Mar 2003
Schneppenheim Reinhard, Budde Ulrich, Oyen Florian, Angerhaus Dorothea, Aumann Volker, Drewke Elke, Hassenpflug Wolf, Häberle Johannes, Kentouche Karim, Kohne Elisabeth, Kurnik Karin, Mueller-Wiefel Dirk, Obser Tobias, Santer René, Sykora Karl-Walter
Abstract excerpt
Thrombotic thrombocytopenic purpura (TTP) is caused by the persistence of the highly reactive high-molecular-weight multimers of von Willebrand factor (VWF) due to deficiency of the specific VWF-cleaving protease (VWF-CP) ADAMTS13, resulting in microangiopathic disease. The acquired form is caused by autoantibodies against VWF-CP, whereas homozygous or compound heterozygous mutations of ADAMTS13 are responsible...
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