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Literature summary extracted from

  • Hughes, C.; McEwan, J.R.; Longair, I.; Hughes, S.; Cohen, H.; Machin, S.; Scully, M.
    Cardiac involvement in acute thrombotic thrombocytopenic purpura: association with troponin T and IgG antibodies to ADAMTS 13 (2009), J. Thromb. Haemost., 7, 529-536.
    View publication on PubMed

Localization

EC Number Localization Comment Organism GeneOntology No. Textmining
3.4.24.87 extracellular
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 Homo sapiens
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Natural Substrates/ Products (Substrates)

EC Number Natural Substrates Organism Comment (Nat. Sub.) Natural Products Comment (Nat. Pro.) Rev. Reac.
3.4.24.87 von Willebrand factor + H2O Homo sapiens cleavage of ultra large von Willebrand multimers into smaller fragments von Willebrand factor fragments
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 ?

Organism

EC Number Organism UniProt Comment Textmining
3.4.24.87 Homo sapiens
-
-
-

Source Tissue

EC Number Source Tissue Comment Organism Textmining
3.4.24.87 plasma
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 Homo sapiens
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Substrates and Products (Substrate)

EC Number Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
3.4.24.87 additional information ADAMTS 13 activity measurement using a collagen binding assay Homo sapiens ?
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 ?
3.4.24.87 von Willebrand factor + H2O cleavage of ultra large von Willebrand multimers into smaller fragments Homo sapiens von Willebrand factor fragments
-
 ?

Synonyms

EC Number Synonyms Comment Organism
3.4.24.87 ADAMTS 13
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 Homo sapiens

General Information

EC Number General Information Comment Organism
3.4.24.87 physiological function ADAMTS 13 is required for the cleavage of ultra large von Willebrand multimers into smaller fragments, as they are released from endothelial cells. A deficiency of ADAMTS 13 or inhibition of its action by antibodies, identified in many cases of acquired thrombotic thrombocytopenic purpura, TTP, results in adhesion and aggregation of platelets to ultra large von Willebrand multimers, small vessel thrombosis, primarily capillaries and arterioles and associated microvascular symptoms. Relationship of ADAMTS 13 activity and IgG antibody to ADAMTS 13 and cardiac presentation, phenotypes, overview. The higher troponin T levels in patients with elevated IgG antibody levels to ADAMTS 13, is associated with an increased acute mortality and morbidity Homo sapiens