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Literature summary for 3.4.24.87 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
Search for more literature for 3.4.24.87

Activating Compound

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

Application

Application Comment Organism
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

Inhibitors Comment Organism Structure
additional information auto-antibodies against ADAMTS13 lead to ADAMTS13 deficiency, which causes e.g. thrombotic thrombocytopenic purpurea, overview Homo sapiens

Localization

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

Metals/Ions

Metals/Ions Comment Organism Structure
Ca2+ required Homo sapiens
Zn2+ required 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
-
 ?
-
 ?

Organism

Organism UniProt Comment Textmining
Homo sapiens
-
-
-

Posttranslational Modification

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

Source Tissue

Source Tissue Comment Organism Textmining
hepatic stellate cell retinoid enriched, i.e. lipocyte, secretes the enzyme Homo sapiens
-
plasma
-
 Homo sapiens
-
podocyte secretes the enzyme Homo sapiens
-

Substrates and Products (Substrate)

Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
von Willebrand factor + H2O
-
 Homo sapiens ?
-
 ?
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

Subunits Comment Organism
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

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

General Information

General Information Comment Organism
additional information acquired deficiencies of ADAMTS13 activity, detailed overview Homo sapiens
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