Mitochondria and Reactive Oxygen Species: The Therapeutic Balance of Powers for Duchenne Muscular Dystrophy
Articolo
Data di Pubblicazione:
2024
Citazione:
Mitochondria and Reactive Oxygen Species: The Therapeutic Balance of Powers for Duchenne Muscular Dystrophy / S.R. Casati, D. Cervia, P. Roux-Biejat, C. Moscheni, C. Perrotta, C. DE PALMA. - In: CELLS. - ISSN 2073-4409. - 13:7(2024 Mar 26), pp. 574.1-574.17. [10.3390/cells13070574]
Abstract:
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.
Tipologia IRIS:
01 - Articolo su periodico
Elenco autori:
S.R. Casati, D. Cervia, P. Roux-Biejat, C. Moscheni, C. Perrotta, C. DE PALMA
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