Protein Variants | Comment | Organism |
---|---|---|
E663A | site-directed mutagenesis, the mutant shows activity similar to the wild-type enzyme with substrate VWF73 peptide | Homo sapiens |
E664A | site-directed mutagenesis, the mutant shows activity similar to the wild-type enzyme with substrate VWF73 peptide | Homo sapiens |
G662A | site-directed mutagenesis, the mutant shows activity similar to the wild-type enzyme with substrate VWF73 peptide | Homo sapiens |
additional information | deletion of amino acid residues Arg659-Glu664 from the ADAMTS13 spacer domain by site-directed mutagenesis results in dramatically reduced proteolytic activity toward von Willebrand factor73 peptides, guanidine-HCl denatured von Willebrand factor, and native von Willebrand factor under fluid shear stress, as well as ultralarge von Willebrand factor on endothelial cells | Homo sapiens |
R659A | site-directed mutagenesis, the mutant shows altered activity compared to the wild-type enzyme with substrate VWF73 peptide | Homo sapiens |
R660A | site-directed mutagenesis, the mutant shows altered activity compared to the wild-type enzyme with substrate VWF73 peptide | Homo sapiens |
Y661A | site-directed mutagenesis, the mutant shows altered activity compared to the wild-type enzyme with substrate VWF73 peptide | Homo sapiens |
KM Value [mM] | KM Value Maximum [mM] | Substrate | Comment | Organism | Structure |
---|---|---|---|---|---|
0.0017 | - |
VWF73 peptide | pH 6.0, 37°C, recombinant Y661A mutant ADAMTS13 | Homo sapiens | |
0.0018 | - |
VWF73 peptide | pH 6.0, 37°C, recombinant wild-type ADAMTS13 | Homo sapiens | |
0.0034 | - |
VWF73 peptide | pH 6.0, 37°C, recombinant E664A mutant ADAMTS13 | Homo sapiens | |
0.0035 | - |
VWF73 peptide | pH 6.0, 37°C, recombinant E663A mutant ADAMTS13 | Homo sapiens | |
0.0049 | - |
VWF73 peptide | pH 6.0, 37°C, recombinant G662A mutant ADAMTS13 | Homo sapiens | |
0.005 | - |
VWF73 peptide | pH 6.0, 37°C, recombinant R660A mutant ADAMTS13 | Homo sapiens | |
0.01 | - |
VWF73 peptide | pH 6.0, 37°C, recombinant R659A mutant ADAMTS13 | Homo sapiens | |
0.022 | - |
VWF73 peptide | pH 6.0, 37°C, recombinant ADAMTS13 6-amino-acid-deletion mutant | Homo sapiens |
Localization | Comment | Organism | GeneOntology No. | Textmining |
---|---|---|---|---|
extracellular | - |
Homo sapiens | - |
- |
Metals/Ions | Comment | Organism | Structure |
---|---|---|---|
Ca2+ | required | Homo sapiens |
Natural Substrates | Organism | Comment (Nat. Sub.) | Natural Products | Comment (Nat. Pro.) | Rev. | Reac. |
---|---|---|---|---|---|---|
additional information | Homo sapiens | removal of newly released ultralarge-von Willebrand factor strings or bundles anchored on endothelial cells by ADAMTS13 occurs rapidly and efficiently in the presence and in the absence of fluid shear stress, suggesting that the cell-bound ultra large-von Willebrand factor polymers may be preferred substrates for ADAMTS13 | ? | - |
? | |
von Willebrand factor + H2O | Homo sapiens | ADAMTS13 cleaves von Willebrand factor at the Tyr1605-Met1606 bond | von Willebrand factor fragments | - |
? |
Organism | UniProt | Comment | Textmining |
---|---|---|---|
Homo sapiens | - |
- |
- |
Source Tissue | Comment | Organism | Textmining |
---|---|---|---|
plasma | - |
Homo sapiens | - |
Substrates | Comment Substrates | Organism | Products | Comment (Products) | Rev. | Reac. |
---|---|---|---|---|---|---|
additional information | removal of newly released ultralarge-von Willebrand factor strings or bundles anchored on endothelial cells by ADAMTS13 occurs rapidly and efficiently in the presence and in the absence of fluid shear stress, suggesting that the cell-bound ultra large-von Willebrand factor polymers may be preferred substrates for ADAMTS13 | Homo sapiens | ? | - |
? | |
von Willebrand factor + H2O | ADAMTS13 cleaves von Willebrand factor at the Tyr1605-Met1606 bond | Homo sapiens | von Willebrand factor fragments | - |
? | |
von Willebrand factor + H2O | ADAMTS13 cleaves von Willebrand factor at the Tyr1605-Met1606 bond. The ADAMTS13 spacer domain is required for cleavage of von Willebrand factor, role of the amino acid residues Arg659, Arg660, and Tyr661 of theADAMTS13 spacer domain in substrate recognition, sequence comparisons and kinetics, detailed overview | Homo sapiens | von Willebrand factor fragments | - |
? | |
VWF73 peptide + H2O | von Willebrand factor-derived peptide substrate | Homo sapiens | ? | - |
? |
Subunits | Comment | Organism |
---|---|---|
More | epitope mapping of anti-ADAMTS13 immunoglobulin G from patients with thrombotic thrombocytopenic purpura and sequence alignment of the ADAMTS13 spacer domains of human, mouse, and zebrafish | Homo sapiens |
Synonyms | Comment | Organism |
---|---|---|
ADAMTS13 | - |
Homo sapiens |
More | ADAMTS13 is a member of a disintegrin and metalloprotease with thrombospondin type 1 repeats, ADAMTS, family | Homo sapiens |
Temperature Optimum [°C] | Temperature Optimum Maximum [°C] | Comment | Organism |
---|---|---|---|
37 | - |
assay at | Homo sapiens |
Turnover Number Minimum [1/s] | Turnover Number Maximum [1/s] | Substrate | Comment | Organism | Structure |
---|---|---|---|---|---|
0.76 | - |
VWF73 peptide | pH 6.0, 37°C, recombinant Y661A mutant ADAMTS13 | Homo sapiens | |
1.09 | - |
VWF73 peptide | pH 6.0, 37°C, recombinant R660A mutant ADAMTS13 | Homo sapiens | |
1.32 | - |
VWF73 peptide | pH 6.0, 37°C, recombinant wild-type ADAMTS13 | Homo sapiens | |
1.43 | - |
VWF73 peptide | pH 6.0, 37°C, recombinant R659A mutant ADAMTS13 | Homo sapiens | |
1.55 | - |
VWF73 peptide | pH 6.0, 37°C, recombinant E663A mutant ADAMTS13 | Homo sapiens | |
1.56 | - |
VWF73 peptide | pH 6.0, 37°C, recombinant ADAMTS13 6-amino-acid-deletion mutant | Homo sapiens | |
1.63 | - |
VWF73 peptide | pH 6.0, 37°C, recombinant E664A mutant ADAMTS13 | Homo sapiens | |
2.05 | - |
VWF73 peptide | pH 6.0, 37°C, recombinant G662A mutant ADAMTS13 | Homo sapiens |
pH Optimum Minimum | pH Optimum Maximum | Comment | Organism |
---|---|---|---|
6 | - |
peptide substrates | Homo sapiens |
7.5 | 8 | multimeric substrate | Homo sapiens |
General Information | Comment | Organism |
---|---|---|
physiological function | the proteolytic cleavage of von Willebrand factor by ADAMTS13 appears to be necessary to eliminate ultralarge von Willebrand factor multimers in circulation, thereby preventing the formation of platelet-rich thrombi in small arterioles and capillaries | Homo sapiens |
kcat/KM Value [1/mMs-1] | kcat/KM Value Maximum [1/mMs-1] | Substrate | Comment | Organism | Structure |
---|---|---|---|---|---|
74 | - |
VWF73 peptide | pH 6.0, 37°C, recombinant ADAMTS13 6-amino-acid-deletion mutant | Homo sapiens | |
140.1 | - |
VWF73 peptide | pH 6.0, 37°C, recombinant R659A mutant ADAMTS13 | Homo sapiens | |
219.2 | - |
VWF73 peptide | pH 6.0, 37°C, recombinant R660A mutant ADAMTS13 | Homo sapiens | |
418.4 | - |
VWF73 peptide | pH 6.0, 37°C, recombinant G662A mutant ADAMTS13 | Homo sapiens | |
444.3 | - |
VWF73 peptide | pH 6.0, 37°C, recombinant E663A mutant ADAMTS13 | Homo sapiens | |
447.9 | - |
VWF73 peptide | pH 6.0, 37°C, recombinant Y661A mutant ADAMTS13 | Homo sapiens | |
487.7 | - |
VWF73 peptide | pH 6.0, 37°C, recombinant E664A mutant ADAMTS13 | Homo sapiens | |
733 | - |
VWF73 peptide | pH 6.0, 37°C, recombinant wild-type ADAMTS13 | Homo sapiens |