Cloned (Comment) | Organism |
---|---|
DNA sequence determination and analysis, transient expression of wild-type and mutants as FLAG-tagged enzymes in HeLa cells | Homo sapiens |
Protein Variants | Comment | Organism |
---|---|---|
C508Y | naturally occuring mutant, no secretion of the enzyme to the plasma, possible defects in secretion pathway, or protein folding and stability | Homo sapiens |
additional information | Q449stop mutant is efficiently secreted, has a MW of 54 kDa, and shows no activity, detection of naturally occuring mutations in a Japanese family with thrombotic thrombocytopenic pupura, overview | Homo sapiens |
P475S | naturally occuring mutant, efficient secretion, reduced activity | Homo sapiens |
Q448E | naturally occuring mutant, efficient secretion, fully active | Homo sapiens |
R268P | naturally occuring mutant, no secretion of the enzyme to the plasma, possible defects in secretion pathway, or protein folding and stability | Homo sapiens |
Localization | Comment | Organism | GeneOntology No. | Textmining |
---|---|---|---|---|
extracellular | plasma | 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 | rapid physiological process that occurs on endothelial surfaces, reduced activity results in thrombotic thrombocytopenic pupura | 2 peptides | - |
? |
Organism | UniProt | Comment | Textmining |
---|---|---|---|
Homo sapiens | - |
- |
- |
Posttranslational Modification | Comment | Organism |
---|---|---|
glycoprotein | N-glycosylation | Homo sapiens |
Source Tissue | Comment | Organism | Textmining |
---|---|---|---|
liver | expression at | Homo sapiens | - |
plasma | - |
Homo sapiens | - |
Substrates | Comment Substrates | Organism | Products | Comment (Products) | Rev. | Reac. |
---|---|---|---|---|---|---|
proteins + H2O | - |
Homo sapiens | peptides | - |
? | |
von Willebrand factor + H2O | large multimeric complexes are reduced to smaller ones, cleavage of peptide bond Tyr842-Met843 | Homo sapiens | 2 peptides | - |
? | |
von Willebrand factor + H2O | rapid physiological process that occurs on endothelial surfaces, reduced activity results in thrombotic thrombocytopenic pupura | Homo sapiens | 2 peptides | - |
? |
Subunits | Comment | Organism |
---|---|---|
? | x * 200000-230000, SDS-PAGE | Homo sapiens |
Synonyms | Comment | Organism |
---|---|---|
ADAMTS13 | - |
Homo sapiens |
M12.241 | Merops-ID | Homo sapiens |
von Willebrand factor-cleaving protease | - |
Homo sapiens |
vWF-cleaving protease | - |
Homo sapiens |
VWF-CP | - |
Homo sapiens |