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Literature summary extracted from

  • Chung, D.W.; Fujikawa, K.
    Processing of von Willebrand Factor by ADAMTS-13 (2002), Biochemistry, 41, 11065-11070.
    View publication on PubMed

Cloned(Commentary)

EC Number Cloned (Comment) Organism
3.4.24.87 gene ADAMTS13, location of chromosome 9q34 Homo sapiens

Protein Variants

EC Number Protein Variants Comment Organism
3.4.24.87 additional information natural mutant is cleaved at peptide bond Tyr1605-Met1606 Homo sapiens

Inhibitors

EC Number Inhibitors Comment Organism Structure
3.4.24.87 1,10-phenanthroline activity can be restored by Ca2+ Homo sapiens
3.4.24.87 EDTA activity can be restored by Ca2+, complete inhibition Homo sapiens
3.4.24.87 EGTA activity can be restored by Ca2+, complete inhibition Homo sapiens
3.4.24.87 additional information no inhibition by iodoacetamide, leupeptin, and serine protease inhibitors DFP, PMSF, aprotinin 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 Ba2+ best activating metal ion Homo sapiens
3.4.24.87 Ca2+ Ca2+ binding site with coordinates Asp173, Cys281, Asp284, and Met249, activating Homo sapiens
3.4.24.87 Mg2+ slightly activating Homo sapiens
3.4.24.87 additional information metalloprotease, contains an essential Zn2+ binding site, no activtion by Zn2+ and Mn2+ Homo sapiens
3.4.24.87 Sr2+ activating 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 the cleavage site is exposed to the enzyme by conformational changes due to shear stress in the plasma, decrease of the multimeric pattern of enzyme and decreased activity results in bone marrow transplant-associated thrombotic microangiopathy, cleavage of peptide bond Tyr842-Met843, large hemostatically active multimers are cleaved to smaller less active forms, increased proteolytic degradation in patients suffering von Willebrand disease typ 2A ?
-
 ?

Organism

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

Purification (Commentary)

EC Number Purification (Comment) Organism
3.4.24.87 to homogeneity 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 proteins + H2O enzyme is involved in thrombotic thrombocytopenic purpura Homo sapiens peptides
-
 ?
3.4.24.87 von Willebrand factor + H2O cleavage site is located in the A2 domain of the substrate, cleavage of peptide bond Tyr842-Met843 Homo sapiens 2 peptides
-
 ?
3.4.24.87 von Willebrand factor + H2O the cleavage site is exposed to the enzyme by conformational changes due to shear stress in the plasma, decrease of the multimeric pattern of enzyme and decreased activity results in bone marrow transplant-associated thrombotic microangiopathy, cleavage of peptide bond Tyr842-Met843, large hemostatically active multimers are cleaved to smaller less active forms, increased proteolytic degradation in patients suffering von Willebrand disease typ 2A Homo sapiens ?
-
 ?

Subunits

EC Number Subunits Comment Organism
3.4.24.87 More structure Homo sapiens

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 Upshaw factor
-
 Homo sapiens
3.4.24.87 van Willebrand factor processing activity
-
 Homo sapiens
3.4.24.87 von Willebrand factor cleaving protease
-
 Homo sapiens
3.4.24.87 VWF cleaving protease
-
 Homo sapiens