Genetic variants in the ADAMTS13 and SUPT3H genes are associated with ADAMTS13 activity [PDF]
Blood, 2015 Key Points We identify rs41314453 as the strongest genetic predictor of ADAMTS13 activity, associated with a decrease of >20%. We present evidence of further independent associations with a common variant in SUPT3H, as well as 5 variants at the ADAMTS13 locus.
Vries, PS +11 more
core +9 more sources
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 +3 more sources
Genetic variants in ADAMTS13 as well as smoking are major determinants of plasma ADAMTS13 levels [PDF]
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
Pharmacogenomics, 2011 The multidomain metalloprotease ADAMTS13 limits thrombus formation via the cleavage of large multimeric forms of von Willebrand factor. Deficiency of functional ADAMTS13 is associated with a number of disease pathologies including thrombotic thrombocytopenic purpura, cardiovascular disease and inflammation.
Sandra Chang, Tseng +1 more
openaire +3 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
doaj +3 more sources
ADAMTS13 in Health and Disease [PDF]
Acta Haematologica, 2009 We raised a set of monoclonal antibodies (mAb) against recombinant human ADAMTS13, constructed the first antigen test fully based on mAbs and compared ADAMTS13 antigen and enzymatic activity levels in a large set of plasma samples collected from different patients and healthy controls.
Hendrik B, Feys +2 more
openaire +3 more sources
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 +4 more sources
The Highs and Lows of ADAMTS13 Activity [PDF]
Journal of Clinical MedicineSevere deficiency of ADAMTS13 (<10 iu/dL) is diagnostic of thrombotic thrombocytopenic purpura (TTP) and leads to accumulation of ultra-large vWF multimers, platelet aggregation, and widespread microthrombi, which can be life-threatening. However, the clinical implications of a low ADAMTS13 activity level are not only important in an acute episode ...
Rebecca J. Shaw +4 more
openaire +3 more sources
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
core +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