Oxidative stress and Nrf2 signaling in McArdle disease

McArdle disease (MD) is a metabolic myopathy due to myophosphorylase deficiency, which leads to a severe limitation in the rate of adenosine triphosphate (ATP) resynthesis. Compensatory flux through the myoadenylate deaminase >>xanthine oxidase pathway should result in higher oxidative stress...

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Bibliographic Details
Published in:Molecular genetics and metabolism 2013-11, Vol.110 (3), p.297-302
Main Authors: Kitaoka, Yu, Ogborn, Daniel I., Nilsson, Mats I., Mocellin, Nicholas J., MacNeil, Lauren G., Tarnopolsky, Mark A.
Format: Article
Language:English
Subjects:
Aldehydes - metabolism
Female
Gene Expression Regulation
Glucosyltransferases - metabolism
Glycogen Storage Disease Type V - genetics
Glycogen Storage Disease Type V - metabolism
Heme Oxygenase-1 - metabolism
Humans
Intracellular Signaling Peptides and Proteins - metabolism
Kelch-Like ECH-Associated Protein 1
Male
McArdle disease
Middle Aged
Mitochondria - metabolism
NAD(P)H Dehydrogenase (Quinone) - metabolism
NF-E2-Related Factor 2 - metabolism
Nuclear factor erythroid 2-related factor 2
Oxidative stress
Oxidative Stress - genetics
Signal Transduction
Transcription, Genetic
Uric Acid - blood
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Summary:McArdle disease (MD) is a metabolic myopathy due to myophosphorylase deficiency, which leads to a severe limitation in the rate of adenosine triphosphate (ATP) resynthesis. Compensatory flux through the myoadenylate deaminase >>xanthine oxidase pathway should result in higher oxidative stress in skeletal muscle; however, oxidative stress and nuclear factor erythroid 2-related factor 2 (Nrf2) mediated antioxidant response cascade in MD patients have not yet been examined. We show that MD patients have elevated muscle protein carbonyls and 4-hydroxynonenal (4-HNE) in comparison with healthy, age and activity matched controls (P
ISSN:1096-7192
1096-7206
DOI:10.1016/j.ymgme.2013.06.022