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Literature summary extracted from

  • Sauna, Z.E.; Okunji, C.; Hunt, R.C.; Gupta, T.; Allen, C.E.; Plum, E.; Blaisdell, A.; Grigoryan, V.; Geetha, S.; Fathke, R.; Soejima, K.; Kimchi-Sarfaty, C.
    Characterization of conformation-sensitive antibodies to ADAMTS13, the von Willebrand cleavage protease (2009), PLoS ONE, 4, e6506.
    View publication on PubMedView publication on EuropePMC

Protein Variants

EC Number Protein Variants Comment Organism
3.4.24.87 P475S the ADAMTS13 mutant shows similar expression but reduced activity compared to the wild-type enzyme, and minimal von Willebrand factor-induced changes in conformation Homo sapiens

Localization

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

Metals/Ions

EC Number Metals/Ions Comment Organism Structure
3.4.24.87 Zn2+ zinc metalloprotease 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 von Willebrand factor + H2O Homo sapiens a catalysis-deficient ADAMTS13 P475S mutant does not show VWF-induced changes in conformation ?
-
 ?

Organism

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

Source Tissue

EC Number Source Tissue Comment Organism Textmining
3.4.24.87 hepatic stellate cell ADAMTS13 is secreted by almost all tissues but primarily by hepatic stellate cells Homo sapiens
-
3.4.24.87 plasma
-
 Homo sapiens
-

Specific Activity [micromol/min/mg]

EC Number Specific Activity Minimum [µmol/min/mg] Specific Activity Maximum [µmol/min/mg] Comment Organism
3.4.24.87 additional information
-
 intracellular and secreted forms of ADAMTS13 show comparable proteolytic activity Homo sapiens

Substrates and Products (Substrate)

EC Number Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
3.4.24.87 von Willebrand factor + H2O a catalysis-deficient ADAMTS13 P475S mutant does not show VWF-induced changes in conformation Homo sapiens ?
-
 ?
3.4.24.87 von Willebrand factor + H2O i.e. VWF, a large glycoprotein secreted by vascular endothelial cells as multimers Homo sapiens ?
-
 ?

Subunits

EC Number Subunits Comment Organism
3.4.24.87 More ADAMTS13 is a multidomain protein Homo sapiens

Synonyms

EC Number Synonyms Comment Organism
3.4.24.87 ADAMTS13
-
 Homo sapiens
3.4.24.87 More ADAMTS13 is a member of the ADAMTS, a disintegrin and metalloproteinase with thrombospondin motifs, family of proteins Homo sapiens
3.4.24.87 von Willebrand cleavage protease
-
 Homo sapiens

General Information

EC Number General Information Comment Organism
3.4.24.87 physiological function ADAMTS13 is a protease that cleaves the von Willebrand factor, VWF, within intact blood vessels under shear stress. ADAMTS13 is implicated in thrombotic thrombocytopenic purpura pathogenesis, overview Homo sapiens