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 +2 more sources
Characterization of ADAMTS13 and von Willebrand factor levels in septic and non-septic ICU patients.
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 +2 more sources
Low ADAMTS13 Activity Correlates with Increased Mortality in COVID-19 Patients
The causes of coagulopathy associated with coronavirus disease 2019 (COVID-19) are poorly understood. We aimed to investigate the relationship between von Willebrand factor (VWF) biomarkers, intravascular hemolysis, coagulation, and organ damage in COVID-
Joseph M. Sweeney+5 more
doaj +2 more sources
Molecular Biology of ADAMTS13 and Diagnostic Utility of ADAMTS13 Proteolytic Activity and Inhibitor Assays [PDF]
ADAMTS13, a reprolysin-like metalloprotease, limits platelet-rich thrombus formation in the small arteries by cleaving von Willebrand factor (vWF) at the Tyr1605-Met1606 peptide bond. Deficiency of plasma ADAMTS13 activity, due to either an inherited or an acquired etiology, may lead to a potentially lethal syndrome, thrombotic thrombocytopenic purpura
Suresh G. Shelat+2 more
openaire +4 more sources
Genetic variants in ADAMTS13 as well as smoking are major determinants of plasma ADAMTS13 levels [PDF]
Key Points Three independent association signals at ADAMTS13 and smoking were identified as major predictors of plasma ADAMTS13 levels. Evidence was presented that 2 nonsynonymous ADAMTS13 variants were driving the variation of plasma ADAMTS13 concentrations.
Chava Kimchi-Sarfaty+9 more
openaire +5 more sources
Background We conducted a prospective observational study for investigating the changes in the 13th member of a disintegrin-like and metalloprotease with thrombospondin type 1 motif (ADAMTS13) and its association with the coagulofibrinolytic response in ...
Hironori Matsumoto+9 more
doaj +2 more sources
A Thrombocytopenic Thrombotic Purpura in a Patient With a Metastatic HER2+ Breast Cancer: Description of a Case Report [PDF]
ABSTRACT Paraneoplastic (p) TTP is a rare syndrome characterized by an immune‐induced, generalized microangiopathy associated with solid or hematological tumors. This case, reporting a patient with a metastatic HER2+ breast cancer and a pTTP, highlights the rarity of this entity, its difficult and challenging diagnosis, and the complexity of its ...
Raffaele Longo+9 more
wiley +2 more sources
Anti-ADAMTS13 Autoantibodies in Immune-Mediated Thrombotic Thrombocytopenic Purpura [PDF]
Autoantibodies 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
Systemic antithrombotic effects of ADAMTS13 [PDF]
The metalloprotease ADAMTS13 (a disintegrin-like and metalloprotease with thrombospondin type I repeats 13) cleaves highly adhesive large von Willebrand factor (VWF) multimers after their release from the endothelium. ADAMTS13 deficiency is linked to a life-threatening disorder, thrombotic thrombocytopenic purpura (TTP), characterized by platelet-rich ...
David G. Motto+8 more
openaire +5 more sources
Efficacy and Safety of Rituximab in Connective Tissue Disease‐Associated Thrombotic Thrombocytopenic Purpura/Thrombotic Microangiopathy [PDF]
ABSTRACT Introduction This study examined the efficacy and safety of Rituximab (RTX) treatment in connective tissue disease (CTD)‐associated thrombocytopenic purpura (TTP) and thrombotic microangiopathy (TMA), using historical controls as comparators.
Naoaki Ohkubo+7 more
wiley +2 more sources