Nature Communications (Aug 2016)
Cell-permeable succinate prodrugs bypass mitochondrial complex I deficiency
- 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
Affiliations
- Johannes K. Ehinger
- Department of Clinical Sciences Lund, Mitochondrial Medicine, Faculty of Medicine, Lund University, BMC A13, 221 84 Lund, Sweden
- Sarah Piel
- Department of Clinical Sciences Lund, Mitochondrial Medicine, Faculty of Medicine, Lund University, BMC A13, 221 84 Lund, Sweden
- Rhonan Ford
- Selcia Ltd, Fyfield Business and Research Park
- Michael Karlsson
- Department of Clinical Sciences Lund, Mitochondrial Medicine, Faculty of Medicine, Lund University, BMC A13, 221 84 Lund, Sweden
- Fredrik Sjövall
- Department of Clinical Sciences Lund, Mitochondrial Medicine, Faculty of Medicine, Lund University, BMC A13, 221 84 Lund, Sweden
- Eleonor Åsander Frostner
- Department of Clinical Sciences Lund, Mitochondrial Medicine, Faculty of Medicine, Lund University, BMC A13, 221 84 Lund, Sweden
- Saori Morota
- Department of Clinical Sciences Lund, Mitochondrial Medicine, Faculty of Medicine, Lund University, BMC A13, 221 84 Lund, Sweden
- Robert W. Taylor
- Wellcome Trust Centre for Mitochondrial Research, Institute of Neuroscience, The Medical School, Newcastle University
- Doug M. Turnbull
- Wellcome Trust Centre for Mitochondrial Research, Institute of Neuroscience, The Medical School, Newcastle University
- Clive Cornell
- Selcia Ltd, Fyfield Business and Research Park
- Steven J. Moss
- Isomerase Therapeutics Ltd, Chesterford Research Park
- Carsten Metzsch
- Department of Clinical Sciences Lund, Anaesthesiology and Intensive Care, Faculty of Medicine, Lund University
- Magnus J. Hansson
- Department of Clinical Sciences Lund, Mitochondrial Medicine, Faculty of Medicine, Lund University, BMC A13, 221 84 Lund, Sweden
- Hans Fliri
- Mitopharm Ltd, Fyfield Business and Research Park
- Eskil Elmér
- Department of Clinical Sciences Lund, Mitochondrial Medicine, Faculty of Medicine, Lund University, BMC A13, 221 84 Lund, Sweden
- DOI
- https://doi.org/10.1038/ncomms12317
- Journal volume & issue
-
Vol. 7,
no. 1
pp. 1 – 8
Abstract
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.
