Natural Substrates | Organism | Comment (Nat. Sub.) | Natural Products | Comment (Nat. Pro.) | Rev. | Reac. |
---|---|---|---|---|---|---|
von Willebrand factor + H2O | Homo sapiens | - |
? | - |
? |
Organism | UniProt | Comment | Textmining |
---|---|---|---|
Homo sapiens | - |
- |
- |
Purification (Comment) | Organism |
---|---|
- |
Homo sapiens |
Source Tissue | Comment | Organism | Textmining |
---|---|---|---|
blood plasma | - |
Homo sapiens | - |
Substrates | Comment Substrates | Organism | Products | Comment (Products) | Rev. | Reac. |
---|---|---|---|---|---|---|
von Willebrand factor + H2O | - |
Homo sapiens | ? | - |
? |
Synonyms | Comment | Organism |
---|---|---|
a disintegrin and metalloprotease with thrombospondin type 1 repeats 13 | - |
Homo sapiens |
ADAMTS13 | - |
Homo sapiens |
von Willebrand factor specific cleaving protease | - |
Homo sapiens |
General Information | Comment | Organism |
---|---|---|
malfunction | a severe functional deficiency of ADAMTS13 (activity lower than 10%) is associated with thrombotic thrombocytopenic purpura | Homo sapiens |
physiological function | multimeric von Willebrand factor is the mediator of both platelet adhesion to the sub-endothelium and platelet aggregation within the microvessels at high shear rates of blood flow. ADAMTS13 regulates von Willebrand factor adhesive capacity by reducing the size of von Willebrand factor multimers | Homo sapiens |