Cloned (Comment) | Organism |
---|---|
gene ADAMTS13, location of chromosome 9q34 | Homo sapiens |
Protein Variants | Comment | Organism |
---|---|---|
additional information | natural mutant is cleaved at peptide bond Tyr1605-Met1606 | Homo sapiens |
Inhibitors | Comment | Organism | Structure |
---|---|---|---|
1,10-phenanthroline | activity can be restored by Ca2+ | Homo sapiens | |
EDTA | activity can be restored by Ca2+, complete inhibition | Homo sapiens | |
EGTA | activity can be restored by Ca2+, complete inhibition | Homo sapiens | |
additional information | no inhibition by iodoacetamide, leupeptin, and serine protease inhibitors DFP, PMSF, aprotinin | Homo sapiens |
Localization | Comment | Organism | GeneOntology No. | Textmining |
---|---|---|---|---|
extracellular | plasma | Homo sapiens | - |
- |
Metals/Ions | Comment | Organism | Structure |
---|---|---|---|
Ba2+ | best activating metal ion | Homo sapiens | |
Ca2+ | Ca2+ binding site with coordinates Asp173, Cys281, Asp284, and Met249, activating | Homo sapiens | |
Mg2+ | slightly activating | Homo sapiens | |
additional information | metalloprotease, contains an essential Zn2+ binding site, no activtion by Zn2+ and Mn2+ | Homo sapiens | |
Sr2+ | activating | 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 | the cleavage site is exposed to the enzyme by conformational changes due to shear stress in the plasma, decrease of the multimeric pattern of enzyme and decreased activity results in bone marrow transplant-associated thrombotic microangiopathy, cleavage of peptide bond Tyr842-Met843, large hemostatically active multimers are cleaved to smaller less active forms, increased proteolytic degradation in patients suffering von Willebrand disease typ 2A | ? | - |
? |
Organism | UniProt | Comment | Textmining |
---|---|---|---|
Homo sapiens | - |
- |
- |
Purification (Comment) | Organism |
---|---|
to homogeneity | Homo sapiens |
Source Tissue | Comment | Organism | Textmining |
---|---|---|---|
plasma | - |
Homo sapiens | - |
Substrates | Comment Substrates | Organism | Products | Comment (Products) | Rev. | Reac. |
---|---|---|---|---|---|---|
proteins + H2O | - |
Homo sapiens | peptides | - |
? | |
proteins + H2O | enzyme is involved in thrombotic thrombocytopenic purpura | Homo sapiens | peptides | - |
? | |
von Willebrand factor + H2O | cleavage site is located in the A2 domain of the substrate, cleavage of peptide bond Tyr842-Met843 | Homo sapiens | 2 peptides | - |
? | |
von Willebrand factor + H2O | the cleavage site is exposed to the enzyme by conformational changes due to shear stress in the plasma, decrease of the multimeric pattern of enzyme and decreased activity results in bone marrow transplant-associated thrombotic microangiopathy, cleavage of peptide bond Tyr842-Met843, large hemostatically active multimers are cleaved to smaller less active forms, increased proteolytic degradation in patients suffering von Willebrand disease typ 2A | Homo sapiens | ? | - |
? |
Subunits | Comment | Organism |
---|---|---|
More | structure | Homo sapiens |
Synonyms | Comment | Organism |
---|---|---|
ADAMTS-13 | - |
Homo sapiens |
M12.241 | Merops-ID | Homo sapiens |
Upshaw factor | - |
Homo sapiens |
van Willebrand factor processing activity | - |
Homo sapiens |
von Willebrand factor cleaving protease | - |
Homo sapiens |
VWF cleaving protease | - |
Homo sapiens |