Protein Variants | Comment | Organism |
---|---|---|
P475S | the ADAMTS13 mutant shows similar expression but reduced activity compared to the wild-type enzyme, and minimal von Willebrand factor-induced changes in conformation | Homo sapiens |
Localization | Comment | Organism | GeneOntology No. | Textmining |
---|---|---|---|---|
extracellular | - |
Homo sapiens | - |
- |
Metals/Ions | Comment | Organism | Structure |
---|---|---|---|
Zn2+ | zinc metalloprotease | Homo sapiens |
Natural Substrates | Organism | Comment (Nat. Sub.) | Natural Products | Comment (Nat. Pro.) | Rev. | Reac. |
---|---|---|---|---|---|---|
von Willebrand factor + H2O | Homo sapiens | a catalysis-deficient ADAMTS13 P475S mutant does not show VWF-induced changes in conformation | ? | - |
? |
Organism | UniProt | Comment | Textmining |
---|---|---|---|
Homo sapiens | - |
- |
- |
Source Tissue | Comment | Organism | Textmining |
---|---|---|---|
hepatic stellate cell | ADAMTS13 is secreted by almost all tissues but primarily by hepatic stellate cells | Homo sapiens | - |
plasma | - |
Homo sapiens | - |
Specific Activity Minimum [µmol/min/mg] | Specific Activity Maximum [µmol/min/mg] | Comment | Organism |
---|---|---|---|
additional information | - |
intracellular and secreted forms of ADAMTS13 show comparable proteolytic activity | Homo sapiens |
Substrates | Comment Substrates | Organism | Products | Comment (Products) | Rev. | Reac. |
---|---|---|---|---|---|---|
von Willebrand factor + H2O | a catalysis-deficient ADAMTS13 P475S mutant does not show VWF-induced changes in conformation | Homo sapiens | ? | - |
? | |
von Willebrand factor + H2O | i.e. VWF, a large glycoprotein secreted by vascular endothelial cells as multimers | Homo sapiens | ? | - |
? |
Subunits | Comment | Organism |
---|---|---|
More | ADAMTS13 is a multidomain protein | Homo sapiens |
Synonyms | Comment | Organism |
---|---|---|
ADAMTS13 | - |
Homo sapiens |
More | ADAMTS13 is a member of the ADAMTS, a disintegrin and metalloproteinase with thrombospondin motifs, family of proteins | Homo sapiens |
von Willebrand cleavage protease | - |
Homo sapiens |
General Information | Comment | Organism |
---|---|---|
physiological function | ADAMTS13 is a protease that cleaves the von Willebrand factor, VWF, within intact blood vessels under shear stress. ADAMTS13 is implicated in thrombotic thrombocytopenic purpura pathogenesis, overview | Homo sapiens |