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

Keywords : 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