Localization | Comment | Organism | GeneOntology No. | Textmining |
---|---|---|---|---|
extracellular | - |
Homo sapiens | - |
- |
Natural Substrates | Organism | Comment (Nat. Sub.) | Natural Products | Comment (Nat. Pro.) | Rev. | Reac. |
---|---|---|---|---|---|---|
von Willebrand factor + H2O | Homo sapiens | cleavage of ultra large von Willebrand multimers into smaller fragments | 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 | ADAMTS 13 activity measurement using a collagen binding assay | Homo sapiens | ? | - |
? | |
von Willebrand factor + H2O | cleavage of ultra large von Willebrand multimers into smaller fragments | Homo sapiens | von Willebrand factor fragments | - |
? |
Synonyms | Comment | Organism |
---|---|---|
ADAMTS 13 | - |
Homo sapiens |
General Information | Comment | Organism |
---|---|---|
physiological function | ADAMTS 13 is required for the cleavage of ultra large von Willebrand multimers into smaller fragments, as they are released from endothelial cells. A deficiency of ADAMTS 13 or inhibition of its action by antibodies, identified in many cases of acquired thrombotic thrombocytopenic purpura, TTP, results in adhesion and aggregation of platelets to ultra large von Willebrand multimers, small vessel thrombosis, primarily capillaries and arterioles and associated microvascular symptoms. Relationship of ADAMTS 13 activity and IgG antibody to ADAMTS 13 and cardiac presentation, phenotypes, overview. The higher troponin T levels in patients with elevated IgG antibody levels to ADAMTS 13, is associated with an increased acute mortality and morbidity | Homo sapiens |