Activating Compound | Comment | Organism | Structure |
---|---|---|---|
additional information | mild denaturation of fluid shear stress increase the hydrolysis of von Willebrand factor | Homo sapiens |
Application | Comment | Organism |
---|---|---|
diagnostics | Potential role of ADAMTS13 as a diagnostic and prognostic marker of disseminated intravascular coagulopathy. ADAMTS13 and the activity of von Willebrand factor are also a marker for development of multiple organ dysfucntion in infectious and non-infectious systemic inflammatory response syndrome | Homo sapiens |
Inhibitors | Comment | Organism | Structure |
---|---|---|---|
additional information | auto-antibodies against ADAMTS13 lead to ADAMTS13 deficiency, which causes e.g. thrombotic thrombocytopenic purpurea, overview | Homo sapiens |
Localization | Comment | Organism | GeneOntology No. | Textmining |
---|---|---|---|---|
extracellular | - |
Homo sapiens | - |
- |
Metals/Ions | Comment | Organism | Structure |
---|---|---|---|
Ca2+ | required | Homo sapiens | |
Zn2+ | 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 | - |
- |
- |
Posttranslational Modification | Comment | Organism |
---|---|---|
proteolytic modification | the pro-peptide is removed during self-activation, which is not required for full enzyme activity | Homo sapiens |
Source Tissue | Comment | Organism | Textmining |
---|---|---|---|
hepatic stellate cell | retinoid enriched, i.e. lipocyte, secretes the enzyme | Homo sapiens | - |
plasma | - |
Homo sapiens | - |
podocyte | secretes the enzyme | Homo sapiens | - |
Substrates | Comment Substrates | Organism | Products | Comment (Products) | Rev. | Reac. |
---|---|---|---|---|---|---|
von Willebrand factor + H2O | - |
Homo sapiens | ? | - |
? | |
von Willebrand factor + H2O | cleaving of ultra-large multimers between residues Tyr842 and Met843 in the central A2 domain, the TSP-1 domain of ADAMTS13 is required for interaction with the extracellular matrix and the substrate, as well as the CUB domains, that are also essential for intracellular trafficking | Homo sapiens | ? | - |
? |
Subunits | Comment | Organism |
---|---|---|
More | the metalloprotease domain is required for activity but not sufficient, the spacer domain is indispensable for activity, and the Cys-rich domain is also involved in activity. Also an intracellular disulfide bond is essential for catalytic activity. ADAMTS13 multidomain structure, overview | Homo sapiens |
Synonyms | Comment | Organism |
---|---|---|
ADAMTS13 | i.e. a disintegrin and metalloprotease with thrombospondin motif | Homo sapiens |
General Information | Comment | Organism |
---|---|---|
additional information | acquired deficiencies of ADAMTS13 activity, detailed overview | Homo sapiens |
physiological function | ADAMTS13 deficiency, due to different reasons, causes thrombotic thrombocytopenic purpurea, which contributes to the prothrombotic coagulation abnormalities preceding organ dysfunction systemic inflammatory response syndrome, overview | Homo sapiens |