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
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Publicado: Nature Portfolio 2021
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Acceso en línea:https://doaj.org/article/6cdac892607d45f6a6225c942b322a42
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spelling oai:doaj.org-article:6cdac892607d45f6a6225c942b322a422021-12-02T15:02:50ZOverexpression of human BAG3P209L in mice causes restrictive cardiomyopathy10.1038/s41467-021-23858-72041-1723https://doaj.org/article/6cdac892607d45f6a6225c942b322a422021-06-01T00:00:00Zhttps://doi.org/10.1038/s41467-021-23858-7https://doaj.org/toc/2041-1723An 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.Kenichi KimuraAstrid OomsKathrin Graf-RiesenMaithreyan KuppusamyAndreas UngerJulia SchuldJan DaerrAchim LotherCaroline GeisenLutz HeinSatoru TakahashiGuang LiWilhelm RöllWilhelm BlochPeter F. M. van der VenWolfgang A. LinkeSean M. WuPitter F. HuesgenJörg HöhfeldDieter O. FürstBernd K. FleischmannMichael HesseNature PortfolioarticleScienceQENNature Communications, Vol 12, Iss 1, Pp 1-17 (2021)
institution DOAJ
collection DOAJ
language EN
topic Science
Q
spellingShingle Science
Q
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
Overexpression of human BAG3P209L in mice causes restrictive cardiomyopathy
description 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.
format article
author 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
author_facet 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
author_sort Kenichi Kimura
title Overexpression of human BAG3P209L in mice causes restrictive cardiomyopathy
title_short Overexpression of human BAG3P209L in mice causes restrictive cardiomyopathy
title_full Overexpression of human BAG3P209L in mice causes restrictive cardiomyopathy
title_fullStr Overexpression of human BAG3P209L in mice causes restrictive cardiomyopathy
title_full_unstemmed Overexpression of human BAG3P209L in mice causes restrictive cardiomyopathy
title_sort overexpression of human bag3p209l in mice causes restrictive cardiomyopathy
publisher Nature Portfolio
publishDate 2021
url https://doaj.org/article/6cdac892607d45f6a6225c942b322a42
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