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Functional muscle impairment in facioscapulohumeral muscular dystrophy is correlated with oxidative stress and mitochondrial dysfunction
Journal article   Open access   Peer reviewed

Functional muscle impairment in facioscapulohumeral muscular dystrophy is correlated with oxidative stress and mitochondrial dysfunction

Ahmed Turki, Maurice Hayot, Gilles Carnac, Fabien Pillard, Emilie Passerieux, Sébastien Bommart, E. Raynaud, Gerald Hugon, Pincemail Joel, Sylvia Pietri, …
Free Radical Biology and Medicine, pp.1068-1079
11/07/2012
DOI: https://doi.org/10.1016/j.freeradbiomed.2012.06.041

Abstract

ROS double homebox 4 gene GSSG T Lim MVC Lipofuscin Facioscapulohumeral muscular dystrophy (FSHD) 4-Hydroxy-2-nonenal Thiobarbituric acid-reactive substances Lipofuscin Oxidative stress Protein oxidation Radical oxygen species Mitochondria dysfunction Antioxidants Abbreviations: FSHD Cu-SOD Facioscapulohumeral muscular dystrophy (FSHD) U1046 HNE TBARS oxidized glutathione reactive oxygen species manganese-dependent superoxide dismutase DUX4 Bâtiment Crastes de Paulet Oxidative stress endurance limit time maximal voluntary contraction thiobarbituric acid-reactive substances 4-hydroxy-2-nonenal 2-MWT MnSOD facioscapulohumeral dystrophy reduced glutathione GSH copper-zinc-dependent superoxide dismutase glutathione reductase Protein oxidation GR Mitochondria dysfunction Radical oxygen species 2 minute walking test n Correspondence to: INSERM Antioxidants
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