Article
Inactivation of ADAMTS13 by plasmin as a potential cause of thrombotic thrombocytopenic purpura.
Journal of thrombosis and haemostasis : JTH - 1 Sept 2010
Feys H B, Vandeputte N, Palla R, Peyvandi F, Peerlinck K, Deckmyn H, Lijnen H R, Vanhoorelbeke K
Abstract excerpt
BACKGROUND: ADAMTS13 deficiency causes accumulation of unusually large von Willebrand factor molecules, which cross-link platelets in the circulation or on the endothelial surface. This process of intravascular agglutination leads to the microangiopathy thrombotic thrombocytopenic purpura (TTP). Most TTP patients have acquired anti-ADAMTS13 autoantibodies that inhibit enzyme function and/or clear it from the...
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