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; Karen Lambert; Alexandra Belayew; Yegor Vassetzky; Raul Juntas-Morales; Jacques Mercier; Dalila Laoudj-Chenivesse

doi:10.1016/j.freeradbiomed.2012.06.041

Journal articles

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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Journal articles

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Life Sciences [q-bio]

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

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

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