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Literature summary extracted from

  • Bowen, D.J.; Collins, P.W.
    An amino acid polymorphism in von Willebrand factor correlates with increased susceptibility to proteolysis by ADAMTS13 (2003), Blood, 103, 941-947.
    View publication on PubMed

Activating Compound

EC Number Activating Compound Comment Organism Structure
3.4.24.87 Ristocetin activates Homo sapiens

Cloned(Commentary)

EC Number Cloned (Comment) Organism
3.4.24.87 DNA sequence determination of natural genetic variants, heterozygous polymorphism at Tyr/Cys1584 and other, overview Homo sapiens

Localization

EC Number Localization Comment Organism GeneOntology No. Textmining
3.4.24.87 extracellular plasma Homo sapiens
-
-

Natural Substrates/ Products (Substrates)

EC Number Natural Substrates Organism Comment (Nat. Sub.) Natural Products Comment (Nat. Pro.) Rev. Reac.
3.4.24.87 proteins + H2O Homo sapiens
-
 peptides
-
 ?
3.4.24.87 von Willebrand factor + H2O Homo sapiens protein from plasma of patients suffering type I von Willebrand disease is more susceptible to proteolysis by the enzyme due to amino acid polymorphism heterozygous at position Tyr/Cys1584, phenotypic parameters, cleavage of the peptide bond Tyr842-Met843 within the A2 domain 2 peptides of 140 kDa and 176 kDa
-
 ?

Organism

EC Number Organism UniProt Comment Textmining
3.4.24.87 Homo sapiens
-
-
-

Source Tissue

EC Number Source Tissue Comment Organism Textmining
3.4.24.87 plasma from patiens with type I von Willebrand disease, i.e. VWD Homo sapiens
-

Substrates and Products (Substrate)

EC Number Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
3.4.24.87 proteins + H2O
-
 Homo sapiens peptides
-
 ?
3.4.24.87 von Willebrand factor + H2O cleavage of the peptide bond Tyr842-Met843 within the A2 domain Homo sapiens 2 peptides of 140 kDa and 176 kDa
-
 ?
3.4.24.87 von Willebrand factor + H2O protein from plasma of patients suffering type I von Willebrand disease is more susceptible to proteolysis by the enzyme due to amino acid polymorphism heterozygous at position Tyr/Cys1584, phenotypic parameters, cleavage of the peptide bond Tyr842-Met843 within the A2 domain Homo sapiens 2 peptides of 140 kDa and 176 kDa
-
 ?

Synonyms

EC Number Synonyms Comment Organism
3.4.24.87 ADAMTS13 i.e. a disintegrin and metalloprotease with thrombospondin repeats Homo sapiens
3.4.24.87 M12.241 Merops-ID Homo sapiens

Temperature Optimum [°C]

EC Number Temperature Optimum [°C] Temperature Optimum Maximum [°C] Comment Organism
3.4.24.87 37
-
 assay at Homo sapiens

pH Optimum

EC Number pH Optimum Minimum pH Optimum Maximum Comment Organism
3.4.24.87 8
-
 assay at Homo sapiens