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Cell-permeable succinate prodrugs bypass mitochondrial complex I deficiency

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
Source :
Nature Communications, Vol 7, Iss 1, Pp 1-8 (2016)
Publication Year :
2016
Publisher :
Nature Portfolio, 2016.

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.

Subjects

Subjects :
Science

Details

Language :
English
ISSN :
20411723
Volume :
7
Issue :
1
Database :
Directory of Open Access Journals
Journal :
Nature Communications
Publication Type :
Academic Journal
Accession number :
edsdoj.065e786aabe24902981472f3415f76d9
Document Type :
article
Full Text :
https://doi.org/10.1038/ncomms12317