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Literature summary extracted from

  • Rayes, J.; Hollestelle, M.J.; Legendre, P.; Marx, I.; de Groot, P.G.; Christophe, O.D.; Lenting, P.J.; Denis, C.V.
    Mutation and ADAMTS13-dependent modulation of disease severity in a mouse model for von Willebrand disease type 2B (2010), Blood, 115, 4870-4877.
    View publication on PubMed

Localization

EC Number Localization Comment Organism GeneOntology No. Textmining
3.4.24.87 extracellular
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 Mus musculus
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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 Mus musculus von Willebrand factor is also susceptible to cleavage by ADAMTS13 when incorporated in a thrombus ?
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 ?

Organism

EC Number Organism UniProt Comment Textmining
3.4.24.87 Mus musculus
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Source Tissue

EC Number Source Tissue Comment Organism Textmining
3.4.24.87 plasma
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 Mus musculus
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Substrates and Products (Substrate)

EC Number Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
3.4.24.87 von Willebrand factor + H2O
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 Mus musculus ?
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 ?
3.4.24.87 von Willebrand factor + H2O von Willebrand factor is also susceptible to cleavage by ADAMTS13 when incorporated in a thrombus Mus musculus ?
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 ?

Synonyms

EC Number Synonyms Comment Organism
3.4.24.87 ADAMTS13 i.e. a disintegrin and metalloprotease with thrombospondin domain 13 Mus musculus

General Information

EC Number General Information Comment Organism
3.4.24.87 additional information ADAMTS13 deficiency results in more and larger circulating platelet aggregates of von Willebrand factor mutants mVWF/R1306Q and mVWF/V1316M, whereas the full multimer range remains present in the mutant mice. The gain-of-function mutations of the von Willebrand factor leads to the Von Willebrand disease-type 2B, VWD-type 2B, resulting in enhanced platelet binding. Clinical manifestations include increased bleeding tendency, loss of large multimers, thrombocytopenia, and circulating platelet aggregates. The VWD-type 2B phenotype depends on the mutations and ADAMTS13, establishment of a mouse model, overview Mus musculus