Evidence for a role of anti-ADAMTS13 autoantibodies despite normal ADAMTS13 activity in recurrent thrombotic thrombocytopenic purpura [PDF]
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
doaj +2 more sources
Genetic variants in ADAMTS13 as well as smoking are major determinants of plasma ADAMTS13 levels
Blood Advances, 2017 : The metalloprotease ADAMTS13 cleaves von Willebrand factor (VWF) in circulating blood, limiting the size of VWF multimers and regulating VWF activity. Abnormal regulation of VWF contributes to bleeding and to thrombotic disorders.
Qianyi Ma +9 more
doaj +2 more sources
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 +2 more sources
Persistence of circulating ADAMTS13-specific immune complexes in patients with acquired thrombotic thrombocytopenic purpura [PDF]
Haematologica, 2014 Anti-ADAMTS13 autoantibodies are the main cause of acquired thrombotic thrombocytopenic purpura. Binding of these antibodies to ADAMTS13 eventually results in the formation of antigen-antibody immune complexes.
Silvia Ferrari +11 more
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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
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Recombinant ADAMTS13 for Hereditary Thrombotic Thrombocytopenic Purpura. [PDF]
, 2022 A 27-year-old patient with a history of severe obstetrical complications and arterial thrombosis received a diagnosis of hereditary thrombotic thrombocytopenic purpura (TTP) due to severe ADAMTS13 deficiency when she presented with an acute episode in ...
Ganter, Michael T +9 more
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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
Mechanism of ADAMTS13 regulation [PDF]
, 2022 Studies demonstrated ADAMTS13 possesses unique properties with a mystifying regulatory mechanism. ADAMTS13’s role is in its proteolytic function to its VWF.
Madarati, Hasam
core +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
Increased plasma von Willebrand factor antigen levels but normal von Willebrand factor cleaving protease (ADAMTS13) activity in preeclampsia. [PDF]
, 2009 The activity of ADAMTS13, the von Willebrand factor (VWF) cleaving protease is low in several conditions, including HELLP (haemolysis, elevated liver enzymes, and low platelet count) syndrome.
Hársfalvi, Jolán +9 more
core +1 more source