Localization | Comment | Organism | GeneOntology No. | Textmining |
---|---|---|---|---|
extracellular | - |
Homo sapiens | - |
- |
Natural Substrates | Organism | Comment (Nat. Sub.) | Natural Products | Comment (Nat. Pro.) | Rev. | Reac. |
---|---|---|---|---|---|---|
von Willebrand factor + H2O | Homo sapiens | specific cleavage of ultra-large von Willebrand factor multimers | ? | - |
? |
Organism | UniProt | Comment | Textmining |
---|---|---|---|
Homo sapiens | - |
- |
- |
Source Tissue | Comment | Organism | Textmining |
---|---|---|---|
plasma | - |
Homo sapiens | - |
Substrates | Comment Substrates | Organism | Products | Comment (Products) | Rev. | Reac. |
---|---|---|---|---|---|---|
von Willebrand factor + H2O | specific cleavage of ultra-large von Willebrand factor multimers | Homo sapiens | ? | - |
? |
Synonyms | Comment | Organism |
---|---|---|
a disintegrin-like and metalloproteinase domain with thrombospondin type I motifs 13 | - |
Homo sapiens |
ADAMTS13 | - |
Homo sapiens |
von Willebrand factor-cleaving metalloprotease | - |
Homo sapiens |
VWF-cleaving metalloprotease | - |
Homo sapiens |
General Information | Comment | Organism |
---|---|---|
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 |