A genetically-engineered von Willebrand disease type 2B mouse model displays defects in hemostasis and inflammation

von Willebrand disease (VWD)-type 2B is characterized by gain-of-function mutations in the von Willebrand factor (VWF) A1-domain, leading to increased affinity for its platelet-receptor, glycoprotein Ibα. We engineered the first knock-in (KI) murine model for VWD-type 2B by introducing the p.V1316M...

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Pubblicato in:Sci Rep
Autori principali: Adam, Frédéric, Casari, Caterina, Prévost, Nicolas, Kauskot, Alexandre, Loubière, Cécile, Legendre, Paulette, Repérant, Christelle, Baruch, Dominique, Rosa, Jean-Philippe, Bryckaert, Marijke, de Groot, Philip G., Christophe, Olivier D., Lenting, Peter J., Denis, Cécile V.
Natura: Artigo
Lingua:Inglês
Pubblicazione: Nature Publishing Group 2016
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Article
Accesso online:https://ncbi.nlm.nih.gov/pmc/articles/PMC4876317/
https://ncbi.nlm.nih.gov/pubmed/27212476
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1038/srep26306
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