Early activation of antioxidant mechanisms in muscle of mutant Cu/Zn-superoxide dismutase-linked amyotrophic lateral sclerosis mice.
A subset of familial ALS cases is associated with missense mutations in the gene encoding Cu/Zn-superoxide dismutase (SOD1), a free radical scavenging enzyme that protects cells against oxidative stress. Overexpression of these ALS-linked mutations confers an unidentified gain of function to the enzyme that triggers a series of neurological disorders characteristic of human ALS. To understand how skeletal muscle may counteract the progression of the disease, we explored the expression of different molecular effectors involved in antioxidant pathways. Our results are strongly indicative of the early and long-lasting activation of a series of molecular effectors thought to act coordinately in preventing the increased oxidative stress characteristic of ALS.
Jokic
Natasa
N
Laboratoire de Signalisations Moléculaires et Neurodégénérescence, Université Louis Pasteur, Faculté de Médecine, 11 rue Humann, 67085 Strasbourg, France.
Di Scala
Franck
F
Dupuis
Luc
L
Rene
Frederique
F
Muller
Andre
A
Gonzalez De Aguilar
Jose-Luis
JL
Loeffler
Jean-Philippe
JP
D000375:Aging; D019943:Amino Acid Substitution; D000818:Animals; D000975:Antioxidants / Q000378:metabolism; D004195:Disease Models, Animal; D005979:Glutathione Peroxidase / Q000235:genetics; D051379:Mice; D016472:Motor Neuron Disease / Q000235:genetics*; D018482:Muscle, Skeletal / Q000201:enzymology*; D018384:Oxidative Stress; D012333:RNA, Messenger / Q000235:genetics; D020133:Reverse Transcriptase Polymerase Chain Reaction; D013482:Superoxide Dismutase / Q000235:genetics* / Q000378:metabolism; D000072105:Superoxide Dismutase-1; D014158:Transcription, Genetic
D000975:Antioxidants; D012333:RNA, Messenger; C000606290:SOD1 protein, human; D005979:Glutathione Peroxidase; C000606291:Sod1 protein, mouse; D013482:Superoxide Dismutase; D000072105:Superoxide Dismutase-1