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Oxidative stress in muscular dystrophy: from generic evidence to specific sources and targets (Articolo in rivista)

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Oxidative stress in muscular dystrophy: from generic evidence to specific sources and targets (Articolo in rivista) (literal)

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Canton, Marcella; Menazza, Sara; Di Lisa, Fabio (2014)
Oxidative stress in muscular dystrophy: from generic evidence to specific sources and targets
in Journal of muscle research and cell motility
(literal)

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1: Department of Biomedical Sciences, University of Padova, Viale G. Colombo 3, 35131 Padua, Italy; 2: Systems Biology Center, NHLBI, NIH, 10 Center drive, Bethesda, MD 20892, USA; 3: Department of Biomedical Sciences, University of Padova, Viale G. Colombo 3, 35131 Padua, Italy; Institute of Neurosciences, CNR, University of Padova, Viale G. Colombo 3, 35131 Padua, Italy (literal)

Titolo

Oxidative stress in muscular dystrophy: from generic evidence to specific sources and targets (literal)

Abstract

Muscular dystrophies (MDs) are a heterogeneous group of diseases that share a common end-point represented by muscular wasting. MDs are caused by mutations in a variety of genes encoding for different molecules, including extracellular matrix, transmembrane and membrane-associated proteins, cytoplasmic enzymes and nuclear proteins. However, it is still to be elucidated how genetic mutations can affect the molecular mechanisms underlying the contractile impairment occurring in these complex pathologies. The intracellular accumulation of reactive oxygen species (ROS) is widely accepted to play a key role in contractile derangements occurring in the different forms of MDs. However, scarce information is available concerning both the most relevant sources of ROS and their major molecular targets. This review focuses on (i) the sources of ROS, with a special emphasis on monoamine oxidase, a mitochondrial enzyme, and (ii) the targets of ROS, highlighting the relevance of the oxidative modification of myofilament proteins. (literal)

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