Journal of Muscle Research and Cell Motility

, Volume 35, Issue 1, pp 23–36

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

Review

DOI: 10.1007/s10974-014-9380-2

Cite this article as:
Canton, M., Menazza, S. & Di Lisa, F. J Muscle Res Cell Motil (2014) 35: 23. doi:10.1007/s10974-014-9380-2

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.

Keywords

Oxidative stressMuscular dystrophyMyofilament proteinsMitochondriaMonoamine oxidase

Copyright information

© Springer International Publishing Switzerland 2014

Authors and Affiliations

  1. 1.Department of Biomedical SciencesUniversity of PadovaPaduaItaly
  2. 2.Systems Biology Center, NHLBINIHBethesdaUSA
  3. 3.Institute of Neurosciences, CNRUniversity of PadovaPaduaItaly