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Literature summary for 3.4.24.87 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
Search for more literature for 3.4.24.87

Cloned(Commentary)

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

Localization

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

Metals/Ions

Metals/Ions Comment Organism Structure
Ca2+ metalloprotease, conserved binding site using Glu83, ASp173, Cys281, Asp284 Homo sapiens
Zn2+ metalloprotease, conserved binding site Homo sapiens

Natural Substrates/ Products (Substrates)

Natural Substrates Organism Comment (Nat. Sub.) Natural Products Comment (Nat. Pro.) Rev. Reac.
proteins + H2O Homo sapiens enzyme is involved in thrombotic thrombocytopenic purpura peptides
-
 ?
von Willebrand factor + H2O Homo sapiens
-
 ?
-
 ?

Organism

Organism UniProt Comment Textmining
Homo sapiens Q76LX8
-
-

Posttranslational Modification

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

Reaction

Reaction Comment Organism Reaction ID
The enzyme cleaves the von Willebrand factor at bond Tyr842-/-Met843 within the A2 domain active site sequence HEXXHXXGXXHD Homo sapiens
a

Source Tissue

Source Tissue Comment Organism Textmining
liver
-
 Homo sapiens
-
plasma
-
 Homo sapiens
-

Substrates and Products (Substrate)

Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
proteins + H2O
-
 Homo sapiens peptides
-
 ?
proteins + H2O enzyme is involved in thrombotic thrombocytopenic purpura Homo sapiens peptides
-
 ?
von Willebrand factor + H2O
-
 Homo sapiens ?
-
 ?

Synonyms

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