LIM kinase/cofilin dysregulation promotes macrothrombocytopenia in severe von Willebrand disease-type 2B

von Willebrand disease type 2B (VWD-type 2B) is characterized by gain-of-function mutations of von Willebrand factor (vWF) that enhance its binding to platelet glycoprotein Ibα and alter the protein’s multimeric structure. Patients with VWD-type 2B display variable extents of bleeding associated wit...

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Опубликовано в: :JCI Insight
Главные авторы: Kauskot, Alexandre, Poirault-Chassac, Sonia, Adam, Frédéric, Muczynski, Vincent, Aymé, Gabriel, Casari, Caterina, Bordet, Jean-Claude, Soukaseum, Christelle, Rothschild, Chantal, Proulle, Valérie, Pietrzyk-Nivau, Audrey, Berrou, Eliane, Christophe, Olivier D., Rosa, Jean-Philippe, Lenting, Peter J., Bryckaert, Marijke, Denis, Cécile V., Baruch, Dominique
Формат: Artigo
Язык:Inglês
Опубликовано: American Society for Clinical Investigation 2016
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Research Article
Online-ссылка:https://ncbi.nlm.nih.gov/pmc/articles/PMC5053155/
https://ncbi.nlm.nih.gov/pubmed/27734030
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1172/jci.insight.88643
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