Cloned (Comment) | Organism |
---|---|
expression of wild-type and mutant ADAMTS13 in HEK-293 cells | Homo sapiens |
Protein Variants | Comment | Organism |
---|---|---|
E663A | site-directed mutagenesis | Homo sapiens |
E664A | site-directed mutagenesis | Homo sapiens |
G662A | site-directed mutagenesis | Homo sapiens |
R659A | site-directed mutagenesis | Homo sapiens |
R660A | site-directed mutagenesis | Homo sapiens |
R660A/Y661A | site-directed mutagenesis | Homo sapiens |
R660A/Y661A/Y665A | site-directed mutagenesis, the ADAMTS13 variant, i.e. ADAMTS13-RYY, shows a 12fold reduced catalytic efficiency arising from over 25fold reduced substrate binding | Homo sapiens |
R660A/Y665A | site-directed mutagenesis | Homo sapiens |
Y658A | site-directed mutagenesis | Homo sapiens |
Y661A | site-directed mutagenesis | Homo sapiens |
Y661A/Y665A | site-directed mutagenesis | Homo sapiens |
Y665A | site-directed mutagenesis | Homo sapiens |
Metals/Ions | Comment | Organism | Structure |
---|---|---|---|
Ca2+ | required | Homo sapiens |
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 |
---|---|
recombinant wild-type and mutant ADAMTS13 from HEK-293 cells by nickel affinity chromatography | Homo sapiens |
Substrates | Comment Substrates | Organism | Products | Comment (Products) | Rev. | Reac. |
---|---|---|---|---|---|---|
additional information | binding specificities of wild-type ADAMTS13 and ADAMTS13-RYY to von Willebrand factor115, von Willebrand factor106, and full-length von Willebrand factor, overview | Homo sapiens | ? | - |
? | |
von Willebrand factor + H2O | - |
Homo sapiens | ? | - |
? | |
von Willebrand factor + H2O | recombinant substrate stably expressed in HEK293 cells, the ADAMTS13 metalloprotease domain cleaves the von Willebrand factor A2 domain at the Y1605-M1606 scissile bond | Homo sapiens | ? | - |
? |
Synonyms | Comment | Organism |
---|---|---|
ADAMTS13 | - |
Homo sapiens |
Temperature Optimum [°C] | Temperature Optimum Maximum [°C] | Comment | Organism |
---|---|---|---|
37 | - |
assay at | Homo sapiens |
pH Optimum Minimum | pH Optimum Maximum | Comment | Organism |
---|---|---|---|
7.8 | - |
assay at | Homo sapiens |
General Information | Comment | Organism |
---|---|---|
additional information | auto-antibodies, directed toward the spacer domain of ADAMTS13 involving region Y658-Y665 , cause ADAMTS13 deficiency and, as a result, acquired thrombotic thrombocytopenic purpura | Homo sapiens |