Any feedback?
Please rate this page
(literature.php)
(0/150)

BRENDA support

Literature summary for 3.4.24.87 extracted from

  • Niiya, M.; Endo, M.; Shang, D.; Zoltick, P.; Muvarak, N.; Cao, W.; Jin, S.; Skipwith, C.; Motto, D.; Flake, A.; Zheng, X.
    Correction of ADAMTS13 deficiency by in utero gene transfer of lentiviral vector encoding ADAMTS13 genes (2009), Mol. Ther., 17, 34-41.
    View publication on PubMedView publication on EuropePMC

Application

Application Comment Organism
medicine correction of ADAMTS13 deficiency by in utero gene transfer of lentiviral vector encoding ADAMTS13 genes Homo sapiens

Cloned(Commentary)

Cloned (Comment) Organism
gene ADMTS13 and mutant variant truncated after the spacer domain, MDTCS, expression in transgenic mice Homo sapiens

Protein Variants

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

Localization Comment Organism GeneOntology No. Textmining
extracellular
-
Homo sapiens
-
-

Natural Substrates/ Products (Substrates)

Natural Substrates Organism Comment (Nat. Sub.) Natural Products Comment (Nat. Pro.) Rev. Reac.
von Willebrand factor + H2O Homo sapiens
-
?
-
?

Organism

Organism UniProt Comment Textmining
Homo sapiens
-
-
-

Source Tissue

Source Tissue Comment Organism Textmining
plasma
-
Homo sapiens
-

Substrates and Products (Substrate)

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

Synonyms Comment Organism
ADAMTS13 i.e a disintegrin and metalloprotease with thrombospondin 13 Homo sapiens

General Information

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