Application | Comment | Organism |
---|---|---|
medicine | correction of ADAMTS13 deficiency by in utero gene transfer of lentiviral vector encoding ADAMTS13 genes | Homo sapiens |
Cloned (Comment) | Organism |
---|---|
gene ADMTS13 and mutant variant truncated after the spacer domain, MDTCS, expression in transgenic mice | Homo sapiens |
Protein Variants | Comment | Organism |
---|---|---|
additional information | construction of a self-inactivating lentiviral vector encoding human full-length ADAMTS13 and a variant truncated after the spacer domain, MDTCS. In utero gene transfer of lentiviral vector encoding ADAMTS13 genes by injection at embryonic days 8 and 14 resulting in detectable plasma proteolytic activity. The mice expressing ADAMTS13 and MDTCS exhibit reduced sizes of von Willebrand factor compared to the Adamts13-/- mice, they show increased survival rates and functional enzyme activity in plasma, overview. The expressed human ADAMTS13 and MDTCS offer systemic protection against arterial thrombosis | Homo sapiens |
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 | - |
? | - |
? |
Organism | UniProt | Comment | Textmining |
---|---|---|---|
Homo sapiens | - |
- |
- |
Source Tissue | Comment | Organism | Textmining |
---|---|---|---|
plasma | - |
Homo sapiens | - |
Substrates | Comment Substrates | Organism | Products | Comment (Products) | Rev. | Reac. |
---|---|---|---|---|---|---|
GST-VWF73 + H2O | labeled von Willebrand factor-derived peptide substrate | Homo sapiens | ? | - |
? | |
von Willebrand factor + H2O | - |
Homo sapiens | ? | - |
? |
Synonyms | Comment | Organism |
---|---|---|
ADAMTS13 | i.e a disintegrin and metalloprotease with thrombospondin 13 | Homo sapiens |
General Information | Comment | Organism |
---|---|---|
additional information | ADAMTS13 deficiency causes thrombotic thrombocytopenic purpura. Correction of ADAMTS13 deficiency by in mice in utero gene transfer of lentiviral vector encoding human ADAMTS13 genes | Homo sapiens |