Ribosomal transcription is regulated by PGC-1alpha and disturbed in Huntington’s disease

Abstract PGC-1α is a versatile inducer of mitochondrial biogenesis and responsive to the changing energy demands of the cell. As mitochondrial ATP production requires proteins that derive from translation products of cytosolic ribosomes, we asked whether PGC-1α directly takes part in ribosomal bioge...

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Autores principales:	Sarah Jesse, Hanna Bayer, Marius C. Alupei, Martina Zügel, Medhanie Mulaw, Francesca Tuorto, Silke Malmsheimer, Karmveer Singh, Jürgen Steinacker, Uwe Schumann, Albert C. Ludolph, Karin Scharffetter-Kochanek, Anke Witting, Patrick Weydt, Sebastian Iben
Formato:	article
Lenguaje:	EN
Publicado:	Nature Portfolio 2017
Materias:	Medicine R Science Q
Acceso en línea:	https://doaj.org/article/2478132ea7814397b8b16d705aad350d
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spelling	oai:doaj.org-article:2478132ea7814397b8b16d705aad350d2021-12-02T11:53:13ZRibosomal transcription is regulated by PGC-1alpha and disturbed in Huntington’s disease10.1038/s41598-017-09148-72045-2322https://doaj.org/article/2478132ea7814397b8b16d705aad350d2017-08-01T00:00:00Zhttps://doi.org/10.1038/s41598-017-09148-7https://doaj.org/toc/2045-2322Abstract PGC-1α is a versatile inducer of mitochondrial biogenesis and responsive to the changing energy demands of the cell. As mitochondrial ATP production requires proteins that derive from translation products of cytosolic ribosomes, we asked whether PGC-1α directly takes part in ribosomal biogenesis. Here, we show that a fraction of cellular PGC-1α localizes to the nucleolus, the site of ribosomal transcription by RNA polymerase I. Upon activation PGC-1α associates with the ribosomal DNA and boosts recruitment of RNA polymerase I and UBF to the rDNA promoter. This induces RNA polymerase I transcription under different stress conditions in cell culture and mouse models as well as in healthy humans and is impaired already in early stages of human Huntington’s disease. This novel molecular link between ribosomal and mitochondrial biogenesis helps to explain sarcopenia and cachexia in diseases of neurodegenerative origin.Sarah JesseHanna BayerMarius C. AlupeiMartina ZügelMedhanie MulawFrancesca TuortoSilke MalmsheimerKarmveer SinghJürgen SteinackerUwe SchumannAlbert C. LudolphKarin Scharffetter-KochanekAnke WittingPatrick WeydtSebastian IbenNature PortfolioarticleMedicineRScienceQENScientific Reports, Vol 7, Iss 1, Pp 1-10 (2017)
institution	DOAJ
collection	DOAJ
language	EN
topic	Medicine R Science Q
spellingShingle	Medicine R Science Q Sarah Jesse Hanna Bayer Marius C. Alupei Martina Zügel Medhanie Mulaw Francesca Tuorto Silke Malmsheimer Karmveer Singh Jürgen Steinacker Uwe Schumann Albert C. Ludolph Karin Scharffetter-Kochanek Anke Witting Patrick Weydt Sebastian Iben Ribosomal transcription is regulated by PGC-1alpha and disturbed in Huntington’s disease
description	Abstract PGC-1α is a versatile inducer of mitochondrial biogenesis and responsive to the changing energy demands of the cell. As mitochondrial ATP production requires proteins that derive from translation products of cytosolic ribosomes, we asked whether PGC-1α directly takes part in ribosomal biogenesis. Here, we show that a fraction of cellular PGC-1α localizes to the nucleolus, the site of ribosomal transcription by RNA polymerase I. Upon activation PGC-1α associates with the ribosomal DNA and boosts recruitment of RNA polymerase I and UBF to the rDNA promoter. This induces RNA polymerase I transcription under different stress conditions in cell culture and mouse models as well as in healthy humans and is impaired already in early stages of human Huntington’s disease. This novel molecular link between ribosomal and mitochondrial biogenesis helps to explain sarcopenia and cachexia in diseases of neurodegenerative origin.
format	article
author	Sarah Jesse Hanna Bayer Marius C. Alupei Martina Zügel Medhanie Mulaw Francesca Tuorto Silke Malmsheimer Karmveer Singh Jürgen Steinacker Uwe Schumann Albert C. Ludolph Karin Scharffetter-Kochanek Anke Witting Patrick Weydt Sebastian Iben
author_facet	Sarah Jesse Hanna Bayer Marius C. Alupei Martina Zügel Medhanie Mulaw Francesca Tuorto Silke Malmsheimer Karmveer Singh Jürgen Steinacker Uwe Schumann Albert C. Ludolph Karin Scharffetter-Kochanek Anke Witting Patrick Weydt Sebastian Iben
author_sort	Sarah Jesse
title	Ribosomal transcription is regulated by PGC-1alpha and disturbed in Huntington’s disease
title_short	Ribosomal transcription is regulated by PGC-1alpha and disturbed in Huntington’s disease
title_full	Ribosomal transcription is regulated by PGC-1alpha and disturbed in Huntington’s disease
title_fullStr	Ribosomal transcription is regulated by PGC-1alpha and disturbed in Huntington’s disease
title_full_unstemmed	Ribosomal transcription is regulated by PGC-1alpha and disturbed in Huntington’s disease
title_sort	ribosomal transcription is regulated by pgc-1alpha and disturbed in huntington’s disease
publisher	Nature Portfolio
publishDate	2017
url	https://doaj.org/article/2478132ea7814397b8b16d705aad350d
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Ribosomal transcription is regulated by PGC-1alpha and disturbed in Huntington’s disease

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