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Literature summary for 3.4.24.87 extracted from

  • Chung, D.W.; Fujikawa, K.
    Processing of von Willebrand Factor by ADAMTS-13 (2002), Biochemistry, 41, 11065-11070.
    View publication on PubMed
Search for more literature for 3.4.24.87

Cloned(Commentary)

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

Protein Variants

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

Inhibitors

Inhibitors Comment Organism Structure
1,10-phenanthroline activity can be restored by Ca2+ Homo sapiens
EDTA activity can be restored by Ca2+, complete inhibition Homo sapiens
EGTA activity can be restored by Ca2+, complete inhibition Homo sapiens
additional information no inhibition by iodoacetamide, leupeptin, and serine protease inhibitors DFP, PMSF, aprotinin Homo sapiens

Localization

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

Metals/Ions

Metals/Ions Comment Organism Structure
Ba2+ best activating metal ion Homo sapiens
Ca2+ Ca2+ binding site with coordinates Asp173, Cys281, Asp284, and Met249, activating Homo sapiens
Mg2+ slightly activating Homo sapiens
additional information metalloprotease, contains an essential Zn2+ binding site, no activtion by Zn2+ and Mn2+ Homo sapiens
Sr2+ activating 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 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

Organism UniProt Comment Textmining
Homo sapiens
-
-
-

Purification (Commentary)

Purification (Comment) Organism
to homogeneity 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
-
 ?
proteins + H2O enzyme is involved in thrombotic thrombocytopenic purpura Homo sapiens peptides
-
 ?
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
-
 ?
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

Subunits Comment Organism
More structure Homo sapiens

Synonyms

Synonyms Comment Organism
ADAMTS-13
-
 Homo sapiens
M12.241 Merops-ID Homo sapiens
Upshaw factor
-
 Homo sapiens
van Willebrand factor processing activity
-
 Homo sapiens
von Willebrand factor cleaving protease
-
 Homo sapiens
VWF cleaving protease
-
 Homo sapiens