Results 1 to 10 of about 12,902 (159)
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 +3 more sources
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
doaj +4 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
doaj +2 more sources
Anti-ADAMTS13 Autoantibodies in Immune-Mediated Thrombotic Thrombocytopenic Purpura [PDF]
AntibodiesAutoantibodies to ADAMTS13 are at the center of pathology of the immune-mediated thrombotic thrombocytopenic purpura. These autoantibodies can be either inhibitory (enzymatic function) or non-inhibitory, resulting in protein depletion.
Michael R. Snyder, Robert W. Maitta
doaj +2 more sources
GC1126A, a novel ADAMTS13 mutein, evades autoantibodies in immune-mediated thrombotic thrombocytopenic purpura [PDF]
Scientific ReportsImmune-mediated thrombotic thrombocytopenic purpura (iTTP) is a rare and life-threatening blood disorder characterized by the formation of blood clots in small blood vessels. It is caused by antibodies targeting the A disintegrin and metalloprotease with
Heechun Kwak +13 more
doaj +2 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
Role of ADAMTS13, VWF and F8 genes in deep vein thrombosis.
PLoS ONE, 2021 BackgroundWe previously described the association between rare ADAMTS13 single nucleotide variants (SNVs) and deep vein thrombosis (DVT). Moreover, DVT patients with at least one rare ADAMTS13 SNV had a lower ADAMTS13 activity than non-carriers.AimsTo ...
Maria Teresa Pagliari +8 more
doaj +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
Platelets, 2023 ADAMTS13 is a plasma metalloprotease with the primary function of cleaving VWF to maintain hemostasis. Circulating ADAMTS13 is in the closed conformation until blood vessel injury triggers a VWF-dependant activation to the open active form of the protein.
Zoe Markham-Lee +2 more
doaj +1 more source
Phenotypic expression of ADAMTS13 in glomerular endothelial cells.
PLoS ONE, 2011 BackgroundADAMTS13 is the physiological von Willebrand factor (VWF)-cleaving protease. The aim of this study was to examine ADAMTS13 expression in kidneys from ADAMTS13 wild-type (Adamts13⁺/⁺) and deficient (Adamts13⁻/⁻) mice and to investigate the ...
Ramesh Tati +8 more
doaj +1 more source