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Literature summary for 3.4.24.87 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
Search for more literature for 3.4.24.87

Localization

Localization Comment Organism GeneOntology No. Textmining
extracellular
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 Mus musculus
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-

Natural Substrates/ Products (Substrates)

Natural Substrates Organism Comment (Nat. Sub.) Natural Products Comment (Nat. Pro.) Rev. Reac.
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

Organism UniProt Comment Textmining
Mus musculus
-
-
-

Source Tissue

Source Tissue Comment Organism Textmining
plasma
-
 Mus musculus
-

Substrates and Products (Substrate)

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

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

General Information

General Information Comment Organism
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