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Literature summary for 3.4.24.87 extracted from

  • Zheng, X.; Nishio, K.; Majerus, E.M.; Sadler, J.E.
    Cleavage of von Willebrand factor requires the spacer domain of the metalloprotease ADAMTS13 (2003), J. Biol. Chem., 278, 30136-30141.
    View publication on PubMed

Cloned(Commentary)

Cloned (Comment) Organism
transient expression of C-terminally tagged enzyme in COS-7 cells or expression in a Sf9 cells/baculovirus expression system, tagged with His6 or V5 epitopes Homo sapiens

Protein Variants

Protein Variants Comment Organism
additional information construction of an enzyme truncated after the metalloprotease domain, which is inactive, addition of the spacer region can restore activity, overview Homo sapiens

Inhibitors

Inhibitors Comment Organism Structure
additional information inhibition of the enzyme by IgG from a patient with idiopathic thrombotic thrombocytopenic pupura Homo sapiens

Localization

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

Metals/Ions

Metals/Ions Comment Organism Structure
additional information divalent metal ion-dependent Homo sapiens

Natural Substrates/ Products (Substrates)

Natural Substrates Organism Comment (Nat. Sub.) Natural Products Comment (Nat. Pro.) Rev. Reac.
proteins + H2O Homo sapiens
-
peptides
-
?
von Willebrand factor + H2O Homo sapiens enzyme deficiency causes lethal thrombotic thrombocytopenic purpura, cleavage of peptide bond Tyr1605-Met1606 resulting in limited platelet accumulation in microvascular thrombi (Tyr842-Met843 within the A2 domain, i.e. Tyr1605-Met1606 in von Willebrand factor UniProt Id P04275) ?
-
?

Organism

Organism UniProt Comment Textmining
Homo sapiens
-
-
-

Reaction

Reaction Comment Organism Reaction ID
The enzyme cleaves the von Willebrand factor at bond Tyr842-/-Met843 within the A2 domain the spacer region is essential, while the C-terminal thrombospondin type 1 motif and the CUB domain are dispensable in vitro 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.
proteins + H2O
-
Homo sapiens peptides
-
?
von Willebrand factor + H2O enzyme deficiency causes lethal thrombotic thrombocytopenic purpura, cleavage of peptide bond Tyr1605-Met1606 resulting in limited platelet accumulation in microvascular thrombi (Tyr842-Met843 within the A2 domain, i.e. Tyr1605-Met1606 in von Willebrand factor UniProt Id P04275) Homo sapiens ?
-
?
von Willebrand factor + H2O large multimeric complexes are reduced to smaller ones, cleavage of peptide bond Tyr842-Met843 in the vWFdomain A2 Homo sapiens 2 peptides of 176 kD and 140 kD
-
?

Synonyms

Synonyms Comment Organism
ADAMTS13
-
Homo sapiens
M12.241 Merops-ID Homo sapiens
von-Willebrand factor cleaving protease
-
Homo sapiens