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Literature summary extracted from

  • Kato, R.; Shinohara, A.; Sato, J.
    ADAMTS13 deficiency, an important cause of thrombocytopenia during pregnancy (2009), Int. J. Obstet. Anesth., 18, 73-77.
    View publication on PubMed

Localization

EC Number Localization Comment Organism GeneOntology No. Textmining
3.4.24.87 extracellular
-
 Homo sapiens
-
-

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 specific cleavage of ultra-large von Willebrand factor multimers ?
-
 ?

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
-
 Homo sapiens
-

Substrates and Products (Substrate)

EC Number Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
3.4.24.87 von Willebrand factor + H2O specific cleavage of ultra-large von Willebrand factor multimers Homo sapiens ?
-
 ?

Synonyms

EC Number Synonyms Comment Organism
3.4.24.87 a disintegrin-like and metalloproteinase domain with thrombospondin type I motifs 13
-
 Homo sapiens
3.4.24.87 ADAMTS13
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 Homo sapiens
3.4.24.87 von Willebrand factor-cleaving metalloprotease
-
 Homo sapiens
3.4.24.87 VWF-cleaving metalloprotease
-
 Homo sapiens

General Information

EC Number General Information Comment Organism
3.4.24.87 physiological function ADAMTS13 is a metalloprotease that limits admission and persistence of unusually large VWF multimers in the circulation, where they form abnormally large thrombi in the microvasculature. Thrombotic thrombocytopenic purpura is caused by congenital ADAMTS13 deficiency, and ADAMTS13 deficiency is an important cause of thrombocytopenia during pregnancy. Thrombotic thrombocytopenic purpura is a rare but life-threatening disease characterized by microangiopathic hemolytic anemia and consumptive thrombocytopenia leading to disseminated microvascular thrombosis Homo sapiens