Search Results - "Deforche, Louis"
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Conformational activation of ADAMTS13
Published in Proceedings of the National Academy of Sciences - PNAS (30-12-2014)“…Significance We show that a CUB–spacer domain interaction impedes exposure of the ADAMTS13 spacer functional exosite, preventing ADAMTS13 from interacting…”
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Allosteric activation of ADAMTS13 by von Willebrand factor
Published in Proceedings of the National Academy of Sciences - PNAS (30-12-2014)“…Significance The blood protein von Willebrand factor (VWF) is required for platelets to stop bleeding at sites of injury, and the metalloprotease ADAMTS13…”
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Generation of Anti-Murine ADAMTS13 Antibodies and Their Application in a Mouse Model for Acquired Thrombotic Thrombocytopenic Purpura
Published in PloS one (01-08-2016)“…Thrombotic thrombocytopenic purpura (TTP) is a life-threatening thrombotic microangiopathy linked to a deficiency in the metalloprotease ADAMTS13. In the…”
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Anti-ADAMTS13 Antibodies and a Novel Heterozygous p.R1177Q Mutation in a Case of Pregnancy-Onset Immune-Mediated Thrombotic Thrombocytopenic Purpura
Published in TH open : companion journal to thrombosis and haemostasis (01-01-2018)“…In this study, we investigated a case of pregnancy-onset thrombotic thrombocytopenic purpura (TTP). The patient had severely decreased ADAMTS13 ( isintegrin nd…”
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The Hunt for the "Minimal" Structure of a Functional ADAMTS13: Study of Deletion Mutations of ADAMTS13 By Small-Angle X-Ray Scattering
Published in Blood (02-12-2016)“…Introduction: ADAMTS13 is a multidomain protein with metalloprotease (M), disintegrin-like (D), thrombospondin-1 (T), Cys-rich (C) and spacer (S) domains,…”
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Phylogenetic Analysis Identifies a Subset of ADAMTS13 Domains That Are Highly Conserved and Essential for Allosteric Regulation
Published in Blood (02-12-2016)“…Introduction: Human ADAMTS13 has metalloprotease (M), disintegrin-like (D), thrombospondin-1 (T), Cys-rich (C) and spacer (S) domains (proximal domains),…”
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FRETS-VWF73 rather than CBA assay reflects ADAMTS13 proteolytic activity in acquired thrombotic thrombocytopenic purpura patients
Published in Thrombosis and haemostasis (01-08-2014)“…Collagen-binding activity (CBA) and FRETS-VWF73 assays are widely adopted methods for the measurement of the plasmatic activity of ADAMTS13, the von Willebrand…”
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