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Literature summary for 3.4.24.87 extracted from
- Unraveling the scissile bond: how ADAMTS13 recognizes and cleaves von Willebrand factor (2011), Blood, 118, 3212-3221.
Inhibitors
| Inhibitors | Comment | Organism | Structure |
|---|---|---|---|
| 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
| Metals/Ions | Comment | Organism | Structure |
|---|---|---|---|
| Ca2+ | Ca2+ ions are also required for enzyme function | Homo sapiens |  |
| Zn2+ | dependent on | Homo sapiens | |
Molecular Weight [Da]
| Molecular Weight [Da] | Molecular Weight Maximum [Da] | Comment | Organism |
|---|---|---|---|
| 180000 | - |
x * 180000, SDS-PAGE | Homo sapiens |
Natural Substrates/ Products (Substrates)
| Natural Substrates | Organism | Comment (Nat. Sub.) | Natural Products | Comment (Nat. Pro.) | Rev. | Reac. |
|---|---|---|---|---|---|---|
| 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
| Organism | UniProt | Comment | Textmining |
|---|---|---|---|
| Homo sapiens | - |
- |
- |
Posttranslational Modification
| Posttranslational Modification | Comment | Organism |
|---|---|---|
| glycoprotein | - |
Homo sapiens |
Source Tissue
| Source Tissue | Comment | Organism | Textmining |
|---|---|---|---|
| hepatic stellate cell | - |
Homo sapiens | - |
| vascular endothelial cell | - |
Homo sapiens | - |
Substrates and Products (Substrate)
| Substrates | Comment Substrates | Organism | Products | Comment (Products) | Rev. | Reac. |
|---|---|---|---|---|---|---|
| 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
| Subunits | Comment | Organism |
|---|---|---|
| ? | x * 180000, SDS-PAGE | Homo sapiens |
Synonyms
| Synonyms | Comment | Organism |
|---|---|---|
| ADAMTS13 | - |
Homo sapiens |
General Information
| General Information | Comment | Organism |
|---|---|---|
| malfunction | ADAMTS13 deficiency is associated with thrombotic thrombocytopenia purpura with microangiopathic hemolytic anemia and thrombocytopenia | Homo sapiens |
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Release 2023.1 (January 2023)
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