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Literature summary for 3.4.24.87 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

Protein Variants Comment Organism
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

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

Metals/Ions

Metals/Ions Comment Organism Structure
Zn2+ zinc metalloprotease Homo sapiens

Natural Substrates/ Products (Substrates)

Natural Substrates Organism Comment (Nat. Sub.) Natural Products Comment (Nat. Pro.) Rev. Reac.
von Willebrand factor + H2O Homo sapiens a catalysis-deficient ADAMTS13 P475S mutant does not show VWF-induced changes in conformation ?
-
?

Organism

Organism UniProt Comment Textmining
Homo sapiens
-
-
-

Source Tissue

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

Specific Activity [micromol/min/mg]

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

Substrates and Products (Substrate)

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

Subunits

Subunits Comment Organism
More ADAMTS13 is a multidomain protein Homo sapiens

Synonyms

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

General Information

General Information Comment Organism
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