Cell-permeable succinate prodrugs bypass mitochondrial complex I deficiency

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.

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

Internet

https://doi.org/10.1038/ncomms12317

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