Overexpression of human BAG3P209L in mice causes restrictive cardiomyopathy

Overexpression of human BAG3P209L in mice causes restrictive cardiomyopathy

An amino acid exchange (P209L) in the human co-chaperone BAG3 gives rise to severe childhood restrictive cardiomyopathy. Here the authors describe humanized transgenic mouse models which phenocopy the disease and provide insight into the pathogenic mechanisms.

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Autores principales: Kenichi Kimura, Astrid Ooms, Kathrin Graf-Riesen, Maithreyan Kuppusamy, Andreas Unger, Julia Schuld, Jan Daerr, Achim Lother, Caroline Geisen, Lutz Hein, Satoru Takahashi, Guang Li, Wilhelm Röll, Wilhelm Bloch, Peter F. M. van der Ven, Wolfgang A. Linke, Sean M. Wu, Pitter F. Huesgen, Jörg Höhfeld, Dieter O. Fürst, Bernd K. Fleischmann, Michael Hesse
Formato: article
Lenguaje:EN
Publicado: Nature Portfolio 2021
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Science
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Acceso en línea:https://doaj.org/article/6cdac892607d45f6a6225c942b322a42
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