Myopathy associated BAG3 mutations lead to protein aggregation by stalling Hsp70 networks

BAG3 is a Hsp70 co-chaperone that is highly expressed in muscles. Here the authors show that several myofibrillar myopathy causing BAG3 mutations are not impaired in Hsp70 binding, but rather impair the ADP-ATP exchange step of the Hsp70 cycle, causing the aggregation of BAG3, Hsp70 and Hsp70 client...

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Autores principales:	Melanie Meister-Broekema, Rebecca Freilich, Chandhuru Jagadeesan, Jennifer N. Rauch, Rocio Bengoechea, William W. Motley, E. F. Elsiena Kuiper, Melania Minoia, Gabriel V. Furtado, Maria A. W. H. van Waarde, Shawn J. Bird, Adriana Rebelo, Stephan Zuchner, Peter Pytel, Steven S. Scherer, Federica F. Morelli, Serena Carra, Conrad C. Weihl, Steven Bergink, Jason E. Gestwicki, Harm H. Kampinga
Formato:	article
Lenguaje:	EN
Publicado:	Nature Portfolio 2018
Materias:	Science Q
Acceso en línea:	https://doaj.org/article/ddf05b7beeda41dab42d91cc0f57bb70
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spelling	oai:doaj.org-article:ddf05b7beeda41dab42d91cc0f57bb702021-12-02T15:33:58ZMyopathy associated BAG3 mutations lead to protein aggregation by stalling Hsp70 networks10.1038/s41467-018-07718-52041-1723https://doaj.org/article/ddf05b7beeda41dab42d91cc0f57bb702018-12-01T00:00:00Zhttps://doi.org/10.1038/s41467-018-07718-5https://doaj.org/toc/2041-1723BAG3 is a Hsp70 co-chaperone that is highly expressed in muscles. Here the authors show that several myofibrillar myopathy causing BAG3 mutations are not impaired in Hsp70 binding, but rather impair the ADP-ATP exchange step of the Hsp70 cycle, causing the aggregation of BAG3, Hsp70 and Hsp70 clients and leading to a collapse of protein homeostasis.Melanie Meister-BroekemaRebecca FreilichChandhuru JagadeesanJennifer N. RauchRocio BengoecheaWilliam W. MotleyE. F. Elsiena KuiperMelania MinoiaGabriel V. FurtadoMaria A. W. H. van WaardeShawn J. BirdAdriana RebeloStephan ZuchnerPeter PytelSteven S. SchererFederica F. MorelliSerena CarraConrad C. WeihlSteven BerginkJason E. GestwickiHarm H. KampingaNature PortfolioarticleScienceQENNature Communications, Vol 9, Iss 1, Pp 1-14 (2018)
institution	DOAJ
collection	DOAJ
language	EN
topic	Science Q
spellingShingle	Science Q Melanie Meister-Broekema Rebecca Freilich Chandhuru Jagadeesan Jennifer N. Rauch Rocio Bengoechea William W. Motley E. F. Elsiena Kuiper Melania Minoia Gabriel V. Furtado Maria A. W. H. van Waarde Shawn J. Bird Adriana Rebelo Stephan Zuchner Peter Pytel Steven S. Scherer Federica F. Morelli Serena Carra Conrad C. Weihl Steven Bergink Jason E. Gestwicki Harm H. Kampinga Myopathy associated BAG3 mutations lead to protein aggregation by stalling Hsp70 networks
description	BAG3 is a Hsp70 co-chaperone that is highly expressed in muscles. Here the authors show that several myofibrillar myopathy causing BAG3 mutations are not impaired in Hsp70 binding, but rather impair the ADP-ATP exchange step of the Hsp70 cycle, causing the aggregation of BAG3, Hsp70 and Hsp70 clients and leading to a collapse of protein homeostasis.
format	article
author	Melanie Meister-Broekema Rebecca Freilich Chandhuru Jagadeesan Jennifer N. Rauch Rocio Bengoechea William W. Motley E. F. Elsiena Kuiper Melania Minoia Gabriel V. Furtado Maria A. W. H. van Waarde Shawn J. Bird Adriana Rebelo Stephan Zuchner Peter Pytel Steven S. Scherer Federica F. Morelli Serena Carra Conrad C. Weihl Steven Bergink Jason E. Gestwicki Harm H. Kampinga
author_facet	Melanie Meister-Broekema Rebecca Freilich Chandhuru Jagadeesan Jennifer N. Rauch Rocio Bengoechea William W. Motley E. F. Elsiena Kuiper Melania Minoia Gabriel V. Furtado Maria A. W. H. van Waarde Shawn J. Bird Adriana Rebelo Stephan Zuchner Peter Pytel Steven S. Scherer Federica F. Morelli Serena Carra Conrad C. Weihl Steven Bergink Jason E. Gestwicki Harm H. Kampinga
author_sort	Melanie Meister-Broekema
title	Myopathy associated BAG3 mutations lead to protein aggregation by stalling Hsp70 networks
title_short	Myopathy associated BAG3 mutations lead to protein aggregation by stalling Hsp70 networks
title_full	Myopathy associated BAG3 mutations lead to protein aggregation by stalling Hsp70 networks
title_fullStr	Myopathy associated BAG3 mutations lead to protein aggregation by stalling Hsp70 networks
title_full_unstemmed	Myopathy associated BAG3 mutations lead to protein aggregation by stalling Hsp70 networks
title_sort	myopathy associated bag3 mutations lead to protein aggregation by stalling hsp70 networks
publisher	Nature Portfolio
publishDate	2018
url	https://doaj.org/article/ddf05b7beeda41dab42d91cc0f57bb70
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Myopathy associated BAG3 mutations lead to protein aggregation by stalling Hsp70 networks

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