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Literature summary extracted from

  • Soejima, K.; Matsumoto, M.; Kokame, K.; Yagi, H.; Ishizashi, H.; Maeda, H.; Nozaki, C.; Miyata, T.; Fujimura, Y.; Nakagaki, T.
    ADAMTS-13 cysteine-rich/spacer domains are functionally essential for von Willebrand factor cleavage (2003), Blood, 102, 3232-3237.
    View publication on PubMed

Cloned(Commentary)

EC Number Cloned (Comment) Organism
3.4.24.87 transient expression of wild-type and mutants in HeLa cells as FLAG-tagged proteins Homo sapiens

Protein Variants

EC Number Protein Variants Comment Organism
3.4.24.87 D500E point mutation in the RGD cysteine-rich domain, unaltered activity compared to the wild-type enzyme Homo sapiens
3.4.24.87 additional information construction of 13 sequential C-terminal truncated mutants, mutants lacking the the cysteine-rich and spacer domain show dramatically reduced or no activity, the other mutants retain their activity, overview Homo sapiens

Inhibitors

EC Number Inhibitors Comment Organism Structure
3.4.24.87 additional information inhibitory autoantibodies from plasma of 3 patients with acquired thrombotic thrombocytopenic purpura, epitope mapping 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 cleavage of the peptide bond Tyr842-Met843 within the A2 domain, enzyme deficiency causes lethal thrombotic thrombocytopenic purpura 2 peptides of 140 kDa and 176 kDa
-
?

Organism

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

Reaction

EC Number Reaction Comment Organism Reaction ID
3.4.24.87 The enzyme cleaves the von Willebrand factor at bond Tyr842-/-Met843 within the A2 domain the cysteine-rich/spacer domains are essential for the proteolytic activity Homo sapiens

Source Tissue

EC Number Source Tissue Comment Organism Textmining
3.4.24.87 plasma
-
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 cleavage of the peptide bond Tyr842-Met843 within the A2 domain, enzyme deficiency causes lethal thrombotic thrombocytopenic purpura Homo sapiens 2 peptides of 140 kDa and 176 kDa
-
?

Synonyms

EC Number Synonyms Comment Organism
3.4.24.87 ADAMTS-13
-
Homo sapiens
3.4.24.87 M12.241 Merops-ID Homo sapiens
3.4.24.87 von Willebrand factor-cleaving protease
-
Homo sapiens
3.4.24.87 VWF-CP
-
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