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.
Main Authors: | 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 |
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Format: | Article |
Language: | English |
Published: |
Nature Publishing Group
2016-08-01
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Series: | Nature Communications |
Online Access: | https://doi.org/10.1038/ncomms12317 |
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