Cell-permeable succinate prodrugs bypass mitochondrial complex I deficiency

Mitochondrial complex I deficiency is the most common respiratory chain defect in mitochondrial disease in children and currently there is no effective treatment. In this study, the authors show that succinate prodrugs can alleviate metabolic decompensation in Leigh syndrome patient fibroblasts.

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Autores principales:	Johannes K. Ehinger, Sarah Piel, Rhonan Ford, Michael Karlsson, Fredrik Sjövall, Eleonor Åsander Frostner, Saori Morota, Robert W. Taylor, Doug M. Turnbull, Clive Cornell, Steven J. Moss, Carsten Metzsch, Magnus J. Hansson, Hans Fliri, Eskil Elmér
Formato:	article
Lenguaje:	EN
Publicado:	Nature Portfolio 2016
Materias:	Science Q
Acceso en línea:	https://doaj.org/article/065e786aabe24902981472f3415f76d9
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spelling	oai:doaj.org-article:065e786aabe24902981472f3415f76d92021-12-02T14:39:50ZCell-permeable succinate prodrugs bypass mitochondrial complex I deficiency10.1038/ncomms123172041-1723https://doaj.org/article/065e786aabe24902981472f3415f76d92016-08-01T00:00:00Zhttps://doi.org/10.1038/ncomms12317https://doaj.org/toc/2041-1723Mitochondrial complex I deficiency is the most common respiratory chain defect in mitochondrial disease in children and currently there is no effective treatment. In this study, the authors show that succinate prodrugs can alleviate metabolic decompensation in Leigh syndrome patient fibroblasts.Johannes K. EhingerSarah PielRhonan FordMichael KarlssonFredrik SjövallEleonor Åsander FrostnerSaori MorotaRobert W. TaylorDoug M. TurnbullClive CornellSteven J. MossCarsten MetzschMagnus J. HanssonHans FliriEskil ElmérNature PortfolioarticleScienceQENNature Communications, Vol 7, Iss 1, Pp 1-8 (2016)
institution	DOAJ
collection	DOAJ
language	EN
topic	Science Q
spellingShingle	Science Q Johannes K. Ehinger Sarah Piel Rhonan Ford Michael Karlsson Fredrik Sjövall Eleonor Åsander Frostner Saori Morota Robert W. Taylor Doug M. Turnbull Clive Cornell Steven J. Moss Carsten Metzsch Magnus J. Hansson Hans Fliri Eskil Elmér Cell-permeable succinate prodrugs bypass mitochondrial complex I deficiency
description	Mitochondrial complex I deficiency is the most common respiratory chain defect in mitochondrial disease in children and currently there is no effective treatment. In this study, the authors show that succinate prodrugs can alleviate metabolic decompensation in Leigh syndrome patient fibroblasts.
format	article
author	Johannes K. Ehinger Sarah Piel Rhonan Ford Michael Karlsson Fredrik Sjövall Eleonor Åsander Frostner Saori Morota Robert W. Taylor Doug M. Turnbull Clive Cornell Steven J. Moss Carsten Metzsch Magnus J. Hansson Hans Fliri Eskil Elmér
author_facet	Johannes K. Ehinger Sarah Piel Rhonan Ford Michael Karlsson Fredrik Sjövall Eleonor Åsander Frostner Saori Morota Robert W. Taylor Doug M. Turnbull Clive Cornell Steven J. Moss Carsten Metzsch Magnus J. Hansson Hans Fliri Eskil Elmér
author_sort	Johannes K. Ehinger
title	Cell-permeable succinate prodrugs bypass mitochondrial complex I deficiency
title_short	Cell-permeable succinate prodrugs bypass mitochondrial complex I deficiency
title_full	Cell-permeable succinate prodrugs bypass mitochondrial complex I deficiency
title_fullStr	Cell-permeable succinate prodrugs bypass mitochondrial complex I deficiency
title_full_unstemmed	Cell-permeable succinate prodrugs bypass mitochondrial complex I deficiency
title_sort	cell-permeable succinate prodrugs bypass mitochondrial complex i deficiency
publisher	Nature Portfolio
publishDate	2016
url	https://doaj.org/article/065e786aabe24902981472f3415f76d9
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Cell-permeable succinate prodrugs bypass mitochondrial complex I deficiency

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