Pathogenicity of Anti-ADAMTS13 Autoantibodies in Acquired Thrombotic Thrombocytopenic Purpura [PDF]
EBioMedicine, 2015 Background: Acquired thrombotic thrombocytopenic purpura (TTP) is an autoimmune disease in which anti-ADAMTS13 autoantibodies cause severe enzyme deficiency.
Mari R. Thomas +3 more
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Haematologica, 2012 Background Severe ADAMTS13 deficiency is a critical component of the pathogenesis of idiopathic thrombotic thrombocytopenic purpura but is found only in about 60% of patients clinically diagnosed with this disease.Design and Methods Over a period of 8 ...
Rahel Froehlich-Zahnd +11 more
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ADAMTS13 in Bothrops lanceolatus snakebite envenoming: Crude venom-induced reduction of in vitro enzymatic activity and clinical correlation in snakebite patients. [PDF]
PLoS Neglected Tropical DiseasesBackgroundEnvenoming by Bothrops lanceolatus, a viperid endemic to Martinique an island in the Lesser Antilles, induces a unique clinical manifestation, i.e., thrombosis. Pathophysiological signaling leading to thrombotic events remains poorly understood.
Jonathan Florentin +8 more
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Open or closed? Understanding the molecular mechanisms and clinical implications of ADAMTS13's conformation [PDF]
HemaSphereBy proteolyzing prothrombotic von Willebrand factor (VWF) multimers, ADAMTS13 (A Disintegrin And Metalloproteinase with ThromboSpondin type‐1 repeats, member 13) ensures balanced hemostasis and prevents microvascular thrombosis. ADAMTS13's conformational
Quintijn Bonnez, Karen Vanhoorelbeke
doaj +2 more sources
Development of a protease-resistant ADAMTS13 to improve stability against proteolytic degradation [PDF]
Blood Advances: Recombinant ADAMTS13 (a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13) was recently approved by the US Food and Drug Administration for the treatment of heritable thrombotic thrombocytopenic purpura, and preclinical ...
Veronica DeYoung +6 more
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Conformational activation of ADAMTS13 [PDF]
Proceedings of the National Academy of Sciences, 2014 A disintegrin and metalloprotease with thrombospondin motifs 13 (ADAMTS13) is a metalloprotease that regulates von Willebrand factor (VWF) function. ADAMTS13-mediated proteolysis is determined by conformational changes in VWF, but also may depend on its ...
Collins, RF +8 more
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ADAMTS13 Deficiency Worsens Colitis and Exogenous ADAMTS13 Administration Decreases Colitis Severity in Mice [PDF]
TH Open, 2017 Background Inflammatory bowel disease (IBD) affects 1.6 million people in the United States. IBD is associated with an increased risk of thrombosis, which rises with disease activity.
Naamah L. Zitomersky +7 more
doaj +3 more sources
Characterization of ADAMTS13 and von Willebrand factor levels in septic and non-septic ICU patients.
PLoS ONE, 2021 Sepsis is a life-threatening disease characterized by excessive host response to infection that can lead to activation of the coagulation system. Von Willebrand Factor (VWF) and ADAMTS13 are important regulators of hemostasis and their dysregulation ...
Kanwal Singh +7 more
doaj +1 more source
The VWF/ADAMTS13 axis in the antiphospholipid syndrome: ADAMTS13 antibodies and ADAMTS13 dysfunction [PDF]
British Journal of Haematology, 2008 SummaryAutoantibodies to ADAMTS13 (a disintegrin‐like and metalloprotease with thrombospondin type I motif, member 13) play an important role in the development of microthrombosis in thrombotic thrombocytopenic purpura (TTP). In severe cases of antiphospholipid syndrome (APS), microthrombosis can occur similar to that seen in TTP, suggesting possible ...
S K, Austin +5 more
openaire +2 more sources
Biology, 2023 Acute-on-chronic liver failure (ACLF) has a high risk of short-term mortality. A disintegrin-like and metalloproteinase with thrombospondin type-1 motifs 13 (ADAMTS13) is a metalloproteinase that specifically cleaves multimeric von Willebrand factor (VWF)
Hiroaki Takaya +12 more
doaj +1 more source