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ADAMTS13 Protein

Known as: ADAMTS-13 Protein, A Disintegrin and Metalloproteinase with Thrombospondin Motifs 13 Protein, ADAMTS13 Protease 
An ADAMTS protease that contains eight thrombospondin (TS) motifs. It cleaves VON WILLEBRAND FACTOR to control vWF-mediated THROMBOSIS. Mutations in… 
National Institutes of Health

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ADAMTS13 geneIn BloodProcess of secretionantagonists & inhibitors

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ADAMTS13 protein, human

Papers overview

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Highly Cited
2008
Highly Cited
2008
Intensive plasma exchange increases a disintegrin and metalloprotease with thrombospondin motifs-13 activity and reverses organ dysfunction in children with thrombocytopenia-associated multiple organ…
Background:Thrombocytopenia-associated multiple organ failure (TAMOF) is a poorly understood syndrome in critically ill children… 
Highly Cited
2008
Highly Cited
2008
Prevalence of the ADAMTS‐13 missense mutation R1060W in late onset adult thrombotic thrombocytopenic purpura
Summary.  Background: Thrombotic thrombocytopenic purpura (TTP) is most commonly associated with deficiency or inhibition of von… 
Highly Cited
2004
Highly Cited
2004
von Willebrand factor cleaving protease (ADAMTS‐13) and ADAMTS‐13 neutralizing autoantibodies in 100 patients with thrombotic thrombocytopenic purpura
The congenital or acquired deficiency of the von Willebrand factor (VWF) cleaving protease, ADAMTS‐13 has been specifically… 
Review
2004
Review
2004
Measurement of von Willebrand factor cleaving protease (ADAMTS‐13): results of an international collaborative study involving 11 methods testing the same set of coded plasmas
Summary.  Background: ADAMTS‐13 is a von Willebrand factor (VFW)‐cleaving protease. Its congenital or acquired deficiency is… 
Highly Cited
2003
Highly Cited
2003
von Willebrand factor cleaving protease and ADAMTS13 mutations in childhood TTP.
Thrombotic thrombocytopenic purpura (TTP) is caused by the persistence of the highly reactive high-molecular-weight multimers of… 
Highly Cited
2003
Highly Cited
2003
Measurement of von Willebrand factor‐cleaving protease (ADAMTS‐13) activity in plasma: a multicenter comparison of different assay methods
Summary.  A severely deficient ADAMTS‐13 activity (<5%) is a key laboratory finding confirming the diagnosis of thrombotic… 
Highly Cited
2002
Highly Cited
2002
Mutations and common polymorphisms in ADAMTS13 gene responsible for von Willebrand factor-cleaving protease activity
von Willebrand factor (VWF) is synthesized primarily in vascular endothelial cells and secreted into the plasma as unusually… 
Highly Cited
2002
Highly Cited
2002
Cloning, expression, and functional characterization of the von Willebrand factor-cleaving protease (ADAMTS13).
Deficient von Willebrand factor (VWF) degradation has been associated with thrombotic thrombocytopenic purpura (TTP). In… 
Highly Cited
2001
Highly Cited
2001
Microangiopathic haemolytic anaemia in metastasizing malignant tumours is not associated with a severe deficiency of the von Willebrand factor‐cleaving protease
Complete deficiency of von Willebrand factor‐cleaving protease (VWF‐cp) has recently been identified as a pathogenetically… 
Highly Cited
1999
Highly Cited
1999
Estimation of the von Willebrand Factor-cleaving Protease in Plasma Using Monoclonal Antibodies to vWF
Summary A protease present in plasma cleaves von Willebrand factor (vWF) at the peptide bond 842Tyr-843Met of the mature subunit… 
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