Simvastatin improves physiological function and protects against muscle degeneration in mdx mice: A novel therapeutic approach for Duchenne muscular dystrophy

Whitehead, Nicholas P.; Kim, M.; Bible, Ken; Adams, Marvin E.; Froehner, Stanley C.

doi:10.1016/j.nmd.2015.06.457

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“…Interestingly, the pharmacological decrease in NOX2 obtained with the administration of simvastatin improved the mdx phenotype by enhancing diaphragm force and reducing fibrosis. These functional improvements were accompanied by autophagy activation and the decline of oxidative stress [95].…”

Section: The Key Role Of the Antioxidant Defense System In Dystrophic...mentioning

confidence: 95%

Mitochondria and Reactive Oxygen Species: The Therapeutic Balance of Powers for Duchenne Muscular Dystrophy

Casati,

Cervia,

Roux-Biejat

et al. 2024

Cells

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Duchenne muscular dystrophy (DMD) is a genetic progressive muscle-wasting disorder that leads to rapid loss of mobility and premature death. The absence of functional dystrophin in DMD patients reduces sarcolemma stiffness and increases contraction damage, triggering a cascade of events leading to muscle cell degeneration, chronic inflammation, and deposition of fibrotic and adipose tissue. Efforts in the last decade have led to the clinical approval of novel drugs for DMD that aim to restore dystrophin function. However, combination therapies able to restore dystrophin expression and target the myriad of cellular events found impaired in dystrophic muscle are desirable. Muscles are higher energy consumers susceptible to mitochondrial defects. Mitochondria generate a significant source of reactive oxygen species (ROS), and they are, in turn, sensitive to proper redox balance. In both DMD patients and animal models there is compelling evidence that mitochondrial impairments have a key role in the failure of energy homeostasis. Here, we highlighted the main aspects of mitochondrial dysfunction and oxidative stress in DMD and discussed the recent findings linked to mitochondria/ROS-targeted molecules as a therapeutic approach. In this respect, dual targeting of both mitochondria and redox homeostasis emerges as a potential clinical option in DMD.

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Section: The Key Role Of the Antioxidant Defense System In Dystrophic...mentioning

confidence: 95%

Mitochondria and Reactive Oxygen Species: The Therapeutic Balance of Powers for Duchenne Muscular Dystrophy

Casati,

Cervia,

Roux-Biejat

et al. 2024

Cells

View full text Add to dashboard Cite

show abstract

Simvastatin improves physiological function and protects against muscle degeneration in mdx mice: A novel therapeutic approach for Duchenne muscular dystrophy

Cited by 1 publication

References 0 publications

Mitochondria and Reactive Oxygen Species: The Therapeutic Balance of Powers for Duchenne Muscular Dystrophy

Mitochondria and Reactive Oxygen Species: The Therapeutic Balance of Powers for Duchenne Muscular Dystrophy

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