Cloned (Comment) | Organism |
---|---|
transient expression of C-terminally tagged enzyme in COS-7 cells or expression in a Sf9 cells/baculovirus expression system, tagged with His6 or V5 epitopes | Homo sapiens |
Protein Variants | Comment | Organism |
---|---|---|
additional information | construction of an enzyme truncated after the metalloprotease domain, which is inactive, addition of the spacer region can restore activity, overview | Homo sapiens |
Inhibitors | Comment | Organism | Structure |
---|---|---|---|
additional information | inhibition of the enzyme by IgG from a patient with idiopathic thrombotic thrombocytopenic pupura | Homo sapiens |
Localization | Comment | Organism | GeneOntology No. | Textmining |
---|---|---|---|---|
extracellular | plasma | Homo sapiens | - |
- |
Metals/Ions | Comment | Organism | Structure |
---|---|---|---|
additional information | divalent metal ion-dependent | Homo sapiens |
Natural Substrates | Organism | Comment (Nat. Sub.) | Natural Products | Comment (Nat. Pro.) | Rev. | Reac. |
---|---|---|---|---|---|---|
proteins + H2O | Homo sapiens | - |
peptides | - |
? | |
von Willebrand factor + H2O | Homo sapiens | enzyme deficiency causes lethal thrombotic thrombocytopenic purpura, cleavage of peptide bond Tyr1605-Met1606 resulting in limited platelet accumulation in microvascular thrombi (Tyr842-Met843 within the A2 domain, i.e. Tyr1605-Met1606 in von Willebrand factor UniProt Id P04275) | ? | - |
? |
Organism | UniProt | Comment | Textmining |
---|---|---|---|
Homo sapiens | - |
- |
- |
Reaction | Comment | Organism | Reaction ID |
---|---|---|---|
The enzyme cleaves the von Willebrand factor at bond Tyr842-/-Met843 within the A2 domain | the spacer region is essential, while the C-terminal thrombospondin type 1 motif and the CUB domain are dispensable in vitro | Homo sapiens |
Source Tissue | Comment | Organism | Textmining |
---|---|---|---|
plasma | - |
Homo sapiens | - |
Substrates | Comment Substrates | Organism | Products | Comment (Products) | Rev. | Reac. |
---|---|---|---|---|---|---|
proteins + H2O | - |
Homo sapiens | peptides | - |
? | |
von Willebrand factor + H2O | enzyme deficiency causes lethal thrombotic thrombocytopenic purpura, cleavage of peptide bond Tyr1605-Met1606 resulting in limited platelet accumulation in microvascular thrombi (Tyr842-Met843 within the A2 domain, i.e. Tyr1605-Met1606 in von Willebrand factor UniProt Id P04275) | Homo sapiens | ? | - |
? | |
von Willebrand factor + H2O | large multimeric complexes are reduced to smaller ones, cleavage of peptide bond Tyr842-Met843 in the vWFdomain A2 | Homo sapiens | 2 peptides of 176 kD and 140 kD | - |
? |
Synonyms | Comment | Organism |
---|---|---|
ADAMTS13 | - |
Homo sapiens |
M12.241 | Merops-ID | Homo sapiens |
von-Willebrand factor cleaving protease | - |
Homo sapiens |