Nature Communications (Jun 2021)
Overexpression of human BAG3P209L in mice causes restrictive cardiomyopathy
- 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
Affiliations
- Kenichi Kimura
- Institute of Physiology I, Life and Brain Center, Medical Faculty, University of Bonn
- Astrid Ooms
- Institute of Physiology I, Life and Brain Center, Medical Faculty, University of Bonn
- Kathrin Graf-Riesen
- Institute of Physiology I, Life and Brain Center, Medical Faculty, University of Bonn
- Maithreyan Kuppusamy
- Central Institute for Engineering, Electronics and Analytics, ZEA-3, Forschungszentrum Jülich
- Andreas Unger
- Institute of Physiology II, University of Münster
- Julia Schuld
- Department of Molecular Cell Biology, Institute for Cell Biology, University of Bonn
- Jan Daerr
- Department of Molecular Cell Biology, Institute for Cell Biology, University of Bonn
- Achim Lother
- Institute of Experimental and Clinical Pharmacology and Toxicology, Division II, Faculty of Medicine, University of Freiburg
- Caroline Geisen
- Institute of Physiology I, Life and Brain Center, Medical Faculty, University of Bonn
- Lutz Hein
- Institute of Experimental and Clinical Pharmacology and Toxicology, Division II, Faculty of Medicine, University of Freiburg
- Satoru Takahashi
- Department of Anatomy and Embryology, Faculty of Medicine, University of Tsukuba
- Guang Li
- Stanford Cardiovascular Institute, Stanford University School of Medicine
- Wilhelm Röll
- Department of Cardiac Surgery, University of Bonn
- Wilhelm Bloch
- German Sport University Cologne, Department of Molecular and Cellular Sport Medicine
- Peter F. M. van der Ven
- Department of Molecular Cell Biology, Institute for Cell Biology, University of Bonn
- Wolfgang A. Linke
- Institute of Physiology II, University of Münster
- Sean M. Wu
- Stanford Cardiovascular Institute, Stanford University School of Medicine
- Pitter F. Huesgen
- Central Institute for Engineering, Electronics and Analytics, ZEA-3, Forschungszentrum Jülich
- Jörg Höhfeld
- Department of Molecular Cell Biology, Institute for Cell Biology, University of Bonn
- Dieter O. Fürst
- Department of Molecular Cell Biology, Institute for Cell Biology, University of Bonn
- Bernd K. Fleischmann
- Institute of Physiology I, Life and Brain Center, Medical Faculty, University of Bonn
- Michael Hesse
- Institute of Physiology I, Life and Brain Center, Medical Faculty, University of Bonn
- DOI
- https://doi.org/10.1038/s41467-021-23858-7
- Journal volume & issue
-
Vol. 12,
no. 1
pp. 1 – 17
Abstract
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.
