Any feedback?
Please rate this page
(literature.php)
(0/150)

BRENDA support

Literature summary extracted from

  • Claus, R.A.; Bockmeyer, C.L.; Sossdorf, M.; Loesche, W.
    The balance between von-Willebrand factor and its cleaving protease ADAMTS13: biomarker in systemic inflammation and development of organ failure? (2010), Curr. Mol. Med., 10, 236-248.
    View publication on PubMed

Activating Compound

EC Number Activating Compound Comment Organism Structure
3.4.24.87 additional information mild denaturation of fluid shear stress increase the hydrolysis of von Willebrand factor Homo sapiens

Application

EC Number Application Comment Organism
3.4.24.87 diagnostics Potential role of ADAMTS13 as a diagnostic and prognostic marker of disseminated intravascular coagulopathy. ADAMTS13 and the activity of von Willebrand factor are also a marker for development of multiple organ dysfucntion in infectious and non-infectious systemic inflammatory response syndrome Homo sapiens

Inhibitors

EC Number Inhibitors Comment Organism Structure
3.4.24.87 additional information auto-antibodies against ADAMTS13 lead to ADAMTS13 deficiency, which causes e.g. thrombotic thrombocytopenic purpurea, overview 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 Ca2+ required Homo sapiens
3.4.24.87 Zn2+ required 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
-
 ?
-
 ?

Organism

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

Posttranslational Modification

EC Number Posttranslational Modification Comment Organism
3.4.24.87 proteolytic modification the pro-peptide is removed during self-activation, which is not required for full enzyme activity Homo sapiens

Source Tissue

EC Number Source Tissue Comment Organism Textmining
3.4.24.87 hepatic stellate cell retinoid enriched, i.e. lipocyte, secretes the enzyme Homo sapiens
-
3.4.24.87 plasma
-
 Homo sapiens
-
3.4.24.87 podocyte secretes the enzyme 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
-
 Homo sapiens ?
-
 ?
3.4.24.87 von Willebrand factor + H2O cleaving of ultra-large multimers between residues Tyr842 and Met843 in the central A2 domain, the TSP-1 domain of ADAMTS13 is required for interaction with the extracellular matrix and the substrate, as well as the CUB domains, that are also essential for intracellular trafficking Homo sapiens ?
-
 ?

Subunits

EC Number Subunits Comment Organism
3.4.24.87 More the metalloprotease domain is required for activity but not sufficient, the spacer domain is indispensable for activity, and the Cys-rich domain is also involved in activity. Also an intracellular disulfide bond is essential for catalytic activity. ADAMTS13 multidomain structure, overview Homo sapiens

Synonyms

EC Number Synonyms Comment Organism
3.4.24.87 ADAMTS13 i.e. a disintegrin and metalloprotease with thrombospondin motif Homo sapiens

General Information

EC Number General Information Comment Organism
3.4.24.87 additional information acquired deficiencies of ADAMTS13 activity, detailed overview Homo sapiens
3.4.24.87 physiological function ADAMTS13 deficiency, due to different reasons, causes thrombotic thrombocytopenic purpurea, which contributes to the prothrombotic coagulation abnormalities preceding organ dysfunction systemic inflammatory response syndrome, overview Homo sapiens