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
Novel cryptic ADAMTS13 epitopes uncover a distinct open ADAMTS13 conformation in immune-mediated TTP [PDF]
HaematologicaOpen ADAMTS13 conformation is gaining clinical interest as a biomarker for diagnosing immune-mediated thrombotic thrombocytopenic purpura (iTTP) and monitoring remission patients for increased relapse risks.
Quintijn Bonnez +13 more
doaj +2 more sources
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
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
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
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
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
Levels of von Willebrand factor antigen and von Willebrand factor cleaving protease (ADAMTS13) activity predict clinical events in chronic heart failure. [PDF]
, 2009 Decreased activity of ADAMTS13, the von Willebrand factor (VWF) cleaving protease, was recently reported in cardiovascular diseases and in hepatic failure.
Borgulya, Gábor +10 more
core +1 more source
AFM Imaging Reveals Multiple Conformational States of ADAMTS13
Journal of Biological Engineering, 2019 Background ADAMTS13 (A disintegrin and metalloprotease with a thrombospondin type 1 motif 13) cleaves Von Willebrand factor (VWF) to regulate its size, thereby preventing aberrant platelet aggregation and thrombus. Deficiency of ADAMTS13 caused by either
Shanshan Yu +6 more
doaj +1 more source