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Oxidative stress and Nrf2 signaling in McArdle disease.

Abstract：

: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 < 0.05). Nuclear abundance of Nrf2 and Nrf2-antioxidant response element (ARE) binding was also higher in MD patients compared with controls (P < 0.05). The expressions of Nrf2 target genes were also higher in MD patients vs. controls. These observations suggest that MD patients experience elevated levels of oxidative stress, and that the Nrf2-mediated antioxidant response cascade is up-regulated in skeletal muscle to compensate.

journal_name

Mol Genet Metab

journal_title

Molecular genetics and metabolism

authors

Kitaoka Y,Ogborn DI,Nilsson MI,Mocellin NJ,MacNeil LG,Tarnopolsky MA

doi

10.1016/j.ymgme.2013.06.022

subject

Has Abstract

pub_date

2013-11-01 00:00:00

pages

297-302

issue

3

eissn

1096-7192

issn

1096-7206

pii

S1096-7192(13)00224-2

journal_volume

110

pub_type

杂志文章

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