Article
Overexpression of human BAG3P209L in mice causes restrictive cardiomyopathy.
Nature communications - 11 Jun 2021
Kimura Kenichi, Ooms Astrid, Graf-Riesen Kathrin, Kuppusamy Maithreyan, Unger Andreas, Schuld Julia, Daerr Jan, Lother Achim, Geisen Caroline, Hein Lutz, Takahashi Satoru, Li Guang, Röll Wilhelm, Bloch Wilhelm, van der Ven Peter F M, Linke Wolfgang A, Wu Sean M, Huesgen Pitter F, Höhfeld Jörg, Fürst Dieter O, Fleischmann Bernd K, Hesse Michael
Abstract excerpt
An amino acid exchange (P209L) in the HSPB8 binding site of the human co-chaperone BAG3 gives rise to severe childhood cardiomyopathy. To phenocopy the disease in mice and gain insight into its mechanisms, we generated humanized transgenic mouse models. Expression of human BAG3P209L-eGFP in mice caused Z-disc disintegration and formation of protein aggregates. This was accompanied by massive fibrosis resulting in...
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