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Literature summary extracted from

  • Zheng, X.; Chung, D.; Takayama, T.K.; Majerus, E.M.; Sadler, J.E.; Fujikawa, K.
    Structure of von Willebrand factor-cleaving protease (ADAMTS13), a metalloprotease involved in thrombotic thrombocytopenic purpura (2001), J. Biol. Chem., 276, 41059-41063.
    View publication on PubMed

Cloned(Commentary)

EC Number Cloned (Comment) Organism
3.4.24.87 DNA sequence determination and analysis Homo sapiens

Localization

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

Metals/Ions

EC Number Metals/Ions Comment Organism Structure
3.4.24.87 Ca2+ metalloprotease, conserved binding site using Glu83, ASp173, Cys281, Asp284 Homo sapiens
3.4.24.87 Zn2+ metalloprotease, conserved binding site 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 enzyme is involved in thrombotic thrombocytopenic purpura peptides
-
 ?
3.4.24.87 von Willebrand factor + H2O Homo sapiens
-
 ?
-
 ?

Organism

EC Number Organism UniProt Comment Textmining
3.4.24.87 Homo sapiens Q76LX8
-
-

Posttranslational Modification

EC Number Posttranslational Modification Comment Organism
3.4.24.87 glycoprotein binding site for alpha-mannosyl residue, 10 potential N-glycosylation sites, Asn-Xaa-Thr/Ser Homo sapiens
3.4.24.87 proteolytic modification made as a zymogen, requires proteolytic activation, possibly intracellularly by furin, cleavage of residues 1-74 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 active site sequence HEXXHXXGXXHD Homo sapiens
a

Source Tissue

EC Number Source Tissue Comment Organism Textmining
3.4.24.87 liver
-
 Homo sapiens
-
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 proteins + H2O enzyme is involved in thrombotic thrombocytopenic purpura Homo sapiens peptides
-
 ?
3.4.24.87 von Willebrand factor + H2O
-
 Homo sapiens ?
-
 ?

Synonyms

EC Number Synonyms Comment Organism
3.4.24.87 ADAMTS13
-
 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 VWFCP
-
 Homo sapiens