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

Ahmed Turki 1 Maurice Hayot 1, 2 Gilles Carnac 1 Fabien Pillard 3 Emilie Passerieux 1 Sébastien Bommart 1, 2 E. Raynaud 1, 2 Gerald Hugon 1 Pincemail Joel 4 Sylvia Pietri 5 Karen Lambert 1 Alexandra Belayew 6 Yegor Vassetzky 7 Raul Juntas-Morales 8 Jacques Mercier 1, 2 Dalila Laoudj-Chenivesse 1
1 PhyMedExp - Physiologie & médecine expérimentale du Cœur et des Muscles [U 1046]
2 CHRU Montpellier - Centre Hospitalier Régional Universitaire [Montpellier]
3 CHRU Toulouse - Centre Hospitalier Régional Universitaire de Toulouse
4 CHU, Liège
5 ICR - Institut de Chimie Radicalaire
6 Service de Biologie Moléculaire, Université de Mons, Belgique
7 IMC (UMR 8126) - Interactions moléculaires et cancer
UMR8126 - Signalisation, noyaux et innovations en cancérologie
8 UM1 - Université Montpellier 1
Keywords : Lipofuscin Protein oxidation Oxidative stress 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 maximal voluntary contraction Cu-SOD 2 minute walking test n Correspondence to: INSERM Facioscapulohumeral muscular dystrophy (FSHD) T Lim MVC 4-hydroxy-2-nonenal 2-MWT HNE endurance limit time thiobarbituric acid-reactive substances reactive oxygen species TBARS oxidized glutathione ROS manganese-dependent superoxide dismutase facioscapulohumeral dystrophy double homebox 4 gene DUX4 GSSG reduced glutathione MnSOD glutathione reductase GSH copper-zinc-dependent superoxide dismutase Mitochondria dysfunction GR Antioxidants Radical oxygen species Bâtiment Crastes de Paulet U1046
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UNIV-AMU | FNCLCC | CNRS | INC-CNRS | IGR | BS | UNIV-MONTPELLIER

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Ahmed Turki, Maurice Hayot, Gilles Carnac, Fabien Pillard, Emilie Passerieux, et al.. Functional muscle impairment in facioscapulohumeral muscular dystrophy is correlated with oxidative stress and mitochondrial dysfunction. Free Radical Biology and Medicine, Elsevier, 2012, pp.1068-1079. ⟨10.1016/j.freeradbiomed.2012.06.041⟩. ⟨hal-01146575⟩

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