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ADAMTS13 Antibody and Inhibitor Assays
2023A finding of an ADAMTS13 (a disintegrin-like and metalloprotease with thrombospondin type 1 motif, member 13) activity level of
Gary W, Moore +2 more
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ADAMTS13 in Health and Disease
Acta Haematologica, 2009We raised a set of monoclonal antibodies (mAb) against recombinant human ADAMTS13, constructed the first antigen test fully based on mAbs and compared ADAMTS13 antigen and enzymatic activity levels in a large set of plasma samples collected from different patients and healthy controls.
Hendrik B, Feys +2 more
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ADAMTS13: origins, applications, and prospects
Transfusion, 2018ADAMTS13 is an enzyme that acts by cleaving prothrombotic von Willebrand factor (VWF) multimers from the vasculature in a highly regulated manner. In pathologic states such as thrombotic thrombocytopenic purpura (TTP) and other thrombotic microangiopathies (TMAs), VWF can bind to the endothelium and form large multimers. As the anchored VWF chains grow,
William E. Plautz +5 more
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Improving on nature: redesigning ADAMTS13
Blood, 2012In this issue of Blood , Jian and coworkers report on a gain-of function variant of ADAMTS13 that is resistant to the autoantibodies responsible for acquired thrombotic thrombocytopenic purpura (TTP).[1][1] Auto-antibodies directed toward ADAMTS13 prohibit cleavage of von Willebrand factor (VWF)
Hovinga, Johanna A. Kremer +1 more
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Blood Reviews, 2013
Thrombotic thrombocytopenic purpura (TTP) is a rare, life-threatening disease characterized by acute episodes of widespread microvascular thrombosis. The discovery that the plasmatic activity of the von Willebrand factor cleaving protease, ADAMTS13, is severely deficient in patients with TTP partially clarified the pathophysiology of the disease ...
L. A. Lotta +3 more
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Thrombotic thrombocytopenic purpura (TTP) is a rare, life-threatening disease characterized by acute episodes of widespread microvascular thrombosis. The discovery that the plasmatic activity of the von Willebrand factor cleaving protease, ADAMTS13, is severely deficient in patients with TTP partially clarified the pathophysiology of the disease ...
L. A. Lotta +3 more
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Variants in ADAMTS13 and Smoking Contribute to Plasma ADAMTS13 Level Variation
Blood, 2014Abstract Homeostatic processing of von Willebrand factor (VWF) in the circulation relies on proteolysis of ultra-large VWF (ULVWF) multimers by the metalloprotease ADAMTS13. Deficiency in ADAMTS13 results in Thrombotic Thrombocytopenic Purpura (TTP) due to the accumulation of thrombogenic ULVWF.
Karl C. Desch +6 more
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Blood, 2005
Comment on Studt et al, page [542][1] Free hemoglobin inhibits ADAMTS13 activity in vitro, demonstrating a potential source of artifact affecting ADAMTS13 activity assays and inhibitor screens. Deficiency of the ADAMTS13 metalloprotease causes thrombotic thrombocytopenic purpura (TTP).
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Comment on Studt et al, page [542][1] Free hemoglobin inhibits ADAMTS13 activity in vitro, demonstrating a potential source of artifact affecting ADAMTS13 activity assays and inhibitor screens. Deficiency of the ADAMTS13 metalloprotease causes thrombotic thrombocytopenic purpura (TTP).
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2015
ADAMTS13 has been identified as the von Willebrand factor-cleaving protease and is deficient in the disorder thrombotic thrombocytopenic purpura. The identification of vascular endothelium as a site of synthesis and secretion of ADAMTS13 has led to studies of the role of ADAMTS13 in angiogenesis.
Manfai Lee, George M. Rodgers
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ADAMTS13 has been identified as the von Willebrand factor-cleaving protease and is deficient in the disorder thrombotic thrombocytopenic purpura. The identification of vascular endothelium as a site of synthesis and secretion of ADAMTS13 has led to studies of the role of ADAMTS13 in angiogenesis.
Manfai Lee, George M. Rodgers
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ADAMTS13: Structure and Function
2015ADAMTS13, a plasma metalloprotease that cleaves von Willebrand factor (VWF), has been shown to have both antithrombotic and anti-inflammatory activities. Severe deficiency of plasma ADAMTS13 activity is a primary cause of thrombotic thrombocytopenic purpura (TTP), a potentially fatal syndrome, but mild to moderate deficiency of plasma ADAMTS13 activity
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