منابع مشابه
Interactions of von Willebrand factor and ADAMTS13 in von Willebrand disease and thrombotic thrombocytopenic purpura.
The function of von Willebrand factor (VWF), a huge multimeric protein and a key factor in platelet dependent primary haemostasis, is regulated by its specific protease ADAMTS13. The ADAMTS13 dependent degradation of VWF to its proteolytic fragments can be visualized as a characteristic so-called triplet structure of individual VWF oligomers by multimer analysis. Lack of VWF high molecular weig...
متن کاملVon Willebrand Factor, ADAMTS13, and the Risk of Mortality
Von Willebrand Factor (VWF) is a multimeric glycoprotein that plays a major role in platelet adhesion and aggregation. Large VWF multimers are highly procoagulant and are cleaved by its metalloprotease ADAMTS13 (A Disintegrin And Metalloprotease with ThromboSpondin motif repeats 13) in smaller, less procoagulant forms. In case of a deficiency of ADAMTS13, large VWF multimers are not cleaved, re...
متن کاملIncreased Von Willebrand factor, decreased ADAMTS13 and thrombocytopenia in melioidosis
BACKGROUND Melioidosis, caused by bioterror treat agent Burkholderia pseudomallei, is an important cause of community-acquired Gram-negative sepsis in Southeast Asia and Northern Australia. New insights into the pathogenesis of melioidosis may help improve treatment and decrease mortality rates from this dreadful disease. We hypothesized that changes in Von Willebrand factor (VWF) function shou...
متن کاملVon Willebrand factor, ADAMTS13, and thrombotic thrombocytopenic purpura
Discoveries during the past decade have revolutionized our understanding of idiopathic thrombotic thrombocytopenic purpura (TTP). Most cases in adults are caused by acquired autoantibodies that inhibit ADAMTS13, a metalloprotease that cleaves von Willebrand factor within nascent platelet-rich thrombi to prevent hemolysis, thrombocytopenia, and tissue infarction. Although approximately 80% of pa...
متن کاملAllosteric activation of ADAMTS13 by von Willebrand factor.
The metalloprotease ADAMTS13 cleaves von Willebrand factor (VWF) within endovascular platelet aggregates, and ADAMTS13 deficiency causes fatal microvascular thrombosis. The proximal metalloprotease (M), disintegrin-like (D), thrombospondin-1 (T), Cys-rich (C), and spacer (S) domains of ADAMTS13 recognize a cryptic site in VWF that is exposed by tensile force. Another seven T and two complement ...
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ژورنال
عنوان ژورنال: Arteriosclerosis, Thrombosis, and Vascular Biology
سال: 2011
ISSN: 1079-5642,1524-4636
DOI: 10.1161/atvbaha.111.223610