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Literature summary extracted from

  • Crawley, J.T.; de Groot, R.; Xiang, Y.; Luken, B.M.; Lane, D.A.
    Unraveling the scissile bond: how ADAMTS13 recognizes and cleaves von Willebrand factor (2011), Blood, 118, 3212-3221.
    View publication on PubMedView publication on EuropePMC

Inhibitors

EC Number Inhibitors Comment Organism Structure
3.4.24.87 additional information protease activity of ADAMTS13 is controlled not by natural inhibitors but by conformational changes in its substrate, which are induced when von Willebrand factor is subject to elevated rheologic shear forces Homo sapiens

Metals/Ions

EC Number Metals/Ions Comment Organism Structure
3.4.24.87 Ca2+ Ca2+ ions are also required for enzyme function Homo sapiens
3.4.24.87 Zn2+ dependent on Homo sapiens

Molecular Weight [Da]

EC Number Molecular Weight [Da] Molecular Weight Maximum [Da] Comment Organism
3.4.24.87 180000
-
 x * 180000, SDS-PAGE 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 von Willebrand factor A2 domain unfolding is required for proteolysis by ADAMTS13, the ADAMTS13 cleavage site is at Tyr1605-Met1606 ?
-
 ?

Organism

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

Posttranslational Modification

EC Number Posttranslational Modification Comment Organism
3.4.24.87 glycoprotein
-
 Homo sapiens

Source Tissue

EC Number Source Tissue Comment Organism Textmining
3.4.24.87 hepatic stellate cell
-
 Homo sapiens
-
3.4.24.87 vascular endothelial cell
-
 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 von Willebrand factor A2 domain unfolding is required for proteolysis by ADAMTS13, the ADAMTS13 cleavage site is at Tyr1605-Met1606 Homo sapiens ?
-
 ?

Subunits

EC Number Subunits Comment Organism
3.4.24.87 ? x * 180000, SDS-PAGE 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 malfunction ADAMTS13 deficiency is associated with thrombotic thrombocytopenia purpura with microangiopathic hemolytic anemia and thrombocytopenia Homo sapiens