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 D. Laoudj-Chenivesse (1)

1 PhyMedExp - Physiologie & médecine expérimentale du Cœur et des Muscles [U 1046]
2 CHRU Montpellier - Centre Hospitalier Régional Universitaire [Montpellier]
3 CHU Toulouse - Centre Hospitalier 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
8 UM1 - Université Montpellier 1

Mots clés: 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

Domaines: Sciences du Vivant [q-bio]

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, 2012, pp.1068-1079. ⟨10.1016/j.freeradbiomed.2012.06.041⟩. ⟨hal-01146575⟩