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Literature summary for 3.4.24.87 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
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Localization

Localization Comment Organism GeneOntology No. Textmining
extracellular
-
 Homo sapiens
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-

Natural Substrates/ Products (Substrates)

Natural Substrates Organism Comment (Nat. Sub.) Natural Products Comment (Nat. Pro.) Rev. Reac.
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

Organism UniProt Comment Textmining
Homo sapiens
-
-
-

Source Tissue

Source Tissue Comment Organism Textmining
plasma
-
 Homo sapiens
-

Substrates and Products (Substrate)

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

Synonyms

Synonyms Comment Organism
ADAMTS 13
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 Homo sapiens

General Information

General Information Comment Organism
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