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Literature summary extracted from

  • Trionfini, P.; Tomasoni, S.; Galbusera, M.; Motto, D.; Longaretti, L.; Corna, D.; Remuzzi, G.; Benigni, A.
    Adenoviral-mediated gene transfer restores plasma ADAMTS13 antigen and activity in ADAMTS13 knockout mice (2009), Gene Ther., 16, 1373-1379.
    View publication on PubMed

Protein Variants

EC Number Protein Variants Comment Organism
3.4.24.87 additional information ability of systemically administered adenovirus encoding human ADAMTS13 to restore the deficient protein in the circulation of Adamts13-/- mice, derived from B/129 wild-type mice. Injection of the adenovirus efficiently transduces the liver, kidney, lung, heart and spleen, resulting in the secretion of ADAMTS13 into plasma, especially from lung and liver, not from brain, overview Homo sapiens

Inhibitors

EC Number Inhibitors Comment Organism Structure
3.4.24.87 EDTA potent inhibitor of the metalloprotease ADAMTS13 Homo sapiens

Localization

EC Number Localization Comment Organism GeneOntology No. Textmining
3.4.24.87 extracellular the enzyme is secreted Homo sapiens
-
-

Metals/Ions

EC Number Metals/Ions Comment Organism Structure
3.4.24.87 Zn2+ required 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 cleavage of ultra-large 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 cleavage of ultra-large multimers Homo sapiens ?
-
 ?

Synonyms

EC Number Synonyms Comment Organism
3.4.24.87 ADAMTS13
-
 Homo sapiens

General Information

EC Number General Information Comment Organism
3.4.24.87 physiological function ADAMTS13 is a plasma metalloprotease that regulates the size of the von Willebrand factor multimers. Genetic or acquired deficiency of ADAMTS13 causes thrombotic thrombocytopenic purpura, TTP Homo sapiens