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Literature summary for 3.4.24.87 extracted from

  • Laje, P.; Shang, D.; Cao, W.; Niiya, M.; Endo, M.; Radu, A.; DeRogatis, N.; Scheiflinger, F.; Zoltick, P.W.; Flake, A.W.; Zheng, X.L.
    Correction of murine ADAMTS13 deficiency by hematopoietic progenitor cell-mediated gene therapy (2009), Blood, 113, 2172-2180.
    View publication on PubMedView publication on EuropePMC
Search for more literature for 3.4.24.87

Cloned(Commentary)

Cloned (Comment) Organism
gene Adamts13, DNA and amino acid sequence determination, construction of self-inactivated lentiviral vector, and expression of functional His-tagged ADAMTS13 cDNA in COS-7 cells Mus musculus

Protein Variants

Protein Variants Comment Organism
additional information plasma ADAMTS13 proteolytic activity on average can be restored in Adamts13-/- mice to approximately 25% of wild-type level by autologous transplantation of hematopoietic progenitor cells transduced ex vivo with a self-inactivating lentiviral vector encoding a full-length murine Adamts13 and an enhanced GFP reporter gene Mus musculus

Localization

Localization Comment Organism GeneOntology No. Textmining
extracellular
-
 Mus musculus
-
-

Metals/Ions

Metals/Ions Comment Organism Structure
Ba2+ activates Mus musculus
Ca2+ activates Mus musculus

Natural Substrates/ Products (Substrates)

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

Organism

Organism UniProt Comment Textmining
Mus musculus
-
 gene Adamts13
-

Source Tissue

Source Tissue Comment Organism Textmining
plasma
-
 Mus musculus
-

Specific Activity [micromol/min/mg]

Specific Activity Minimum [µmol/min/mg] Specific Activity Maximum [µmol/min/mg] Comment Organism
additional information
-
 determination of ADAMTS13 antigen and proteolytic activity in murine plasma, overview Mus musculus

Substrates and Products (Substrate)

Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
additional information assay on the fluorogenic substrate FRETS-vWF73 Mus musculus ?
-
 ?
von Willebrand factor + H2O
-
 Mus musculus ?
-
 ?
von Willebrand factor + H2O substrate of human origin Mus musculus ?
-
 ?

Synonyms

Synonyms Comment Organism
ADAMTS13
-
 Mus musculus

Temperature Optimum [°C]

Temperature Optimum [°C] Temperature Optimum Maximum [°C] Comment Organism
37
-
 assay at Mus musculus

pH Optimum

pH Optimum Minimum pH Optimum Maximum Comment Organism
7.5 8 assay at Mus musculus

General Information

General Information Comment Organism
additional information genetic or acquired deficiency of plasma ADAMTS13 activity leads to a potentially fatal syndrome, thrombotic thrombocytopenic purpura, TTP Mus musculus