Redox Homeostasis in Muscular Dystrophies
In recent years, growing evidence has suggested a prominent role of oxidative stress in the pathophysiology of several early- and adult-onset muscle disorders, although effective antioxidant treatments are still lacking. Oxidative stress causes cell damage by affecting protein function, membrane str...
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| Format: | Article |
| Language: | English |
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MDPI AG
2021-06-01
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| Series: | Cells |
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| Online Access: | https://www.mdpi.com/2073-4409/10/6/1364 |
| _version_ | 1797531713469939712 |
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| author | Nicola Mosca Sara Petrillo Sara Bortolani Mauro Monforte Enzo Ricci Fiorella Piemonte Giorgio Tasca |
| author_facet | Nicola Mosca Sara Petrillo Sara Bortolani Mauro Monforte Enzo Ricci Fiorella Piemonte Giorgio Tasca |
| author_sort | Nicola Mosca |
| collection | DOAJ |
| description | In recent years, growing evidence has suggested a prominent role of oxidative stress in the pathophysiology of several early- and adult-onset muscle disorders, although effective antioxidant treatments are still lacking. Oxidative stress causes cell damage by affecting protein function, membrane structure, lipid metabolism, and DNA integrity, thus interfering with skeletal muscle homeostasis and functionality. Some features related to oxidative stress, such as chronic inflammation, defective regeneration, and mitochondrial damage are shared among most muscular dystrophies, and Nrf2 has been shown to be a central player in antagonizing redox imbalance in several of these disorders. However, the exact mechanisms leading to overproduction of reactive oxygen species and deregulation in the cellular antioxidants system seem to be, to a large extent, disease-specific, and the clarification of these mechanisms in vivo in humans is the cornerstone for the development of targeted antioxidant therapies, which will require testing in appropriately designed clinical trials. |
| first_indexed | 2024-03-10T10:48:37Z |
| format | Article |
| id | doaj.art-3d096f0eaae14d3bbda6d6ffc35ac25b |
| institution | Directory Open Access Journal |
| issn | 2073-4409 |
| language | English |
| last_indexed | 2024-03-10T10:48:37Z |
| publishDate | 2021-06-01 |
| publisher | MDPI AG |
| record_format | Article |
| series | Cells |
| spelling | doaj.art-3d096f0eaae14d3bbda6d6ffc35ac25b2023-11-21T22:25:12ZengMDPI AGCells2073-44092021-06-01106136410.3390/cells10061364Redox Homeostasis in Muscular DystrophiesNicola Mosca0Sara Petrillo1Sara Bortolani2Mauro Monforte3Enzo Ricci4Fiorella Piemonte5Giorgio Tasca6Unità Operativa Complessa di Neurologia, Fondazione Policlinico Universitario A. Gemelli IRCCS, 00168 Roma, ItalyUnit of Muscular and Neurodegenerative Diseases, Ospedale Pediatrico Bambino Gesù, IRCCS, 00146 Rome, ItalyUnità Operativa Complessa di Neurologia, Fondazione Policlinico Universitario A. Gemelli IRCCS, 00168 Roma, ItalyUnità Operativa Complessa di Neurologia, Fondazione Policlinico Universitario A. Gemelli IRCCS, 00168 Roma, ItalyUnità Operativa Complessa di Neurologia, Fondazione Policlinico Universitario A. Gemelli IRCCS, 00168 Roma, ItalyUnit of Muscular and Neurodegenerative Diseases, Ospedale Pediatrico Bambino Gesù, IRCCS, 00146 Rome, ItalyUnità Operativa Complessa di Neurologia, Fondazione Policlinico Universitario A. Gemelli IRCCS, 00168 Roma, ItalyIn recent years, growing evidence has suggested a prominent role of oxidative stress in the pathophysiology of several early- and adult-onset muscle disorders, although effective antioxidant treatments are still lacking. Oxidative stress causes cell damage by affecting protein function, membrane structure, lipid metabolism, and DNA integrity, thus interfering with skeletal muscle homeostasis and functionality. Some features related to oxidative stress, such as chronic inflammation, defective regeneration, and mitochondrial damage are shared among most muscular dystrophies, and Nrf2 has been shown to be a central player in antagonizing redox imbalance in several of these disorders. However, the exact mechanisms leading to overproduction of reactive oxygen species and deregulation in the cellular antioxidants system seem to be, to a large extent, disease-specific, and the clarification of these mechanisms in vivo in humans is the cornerstone for the development of targeted antioxidant therapies, which will require testing in appropriately designed clinical trials.https://www.mdpi.com/2073-4409/10/6/1364muscular dystrophiesFSHDoxidative stressreactive oxygen species (ROS)antioxidantsNrf2 |
| spellingShingle | Nicola Mosca Sara Petrillo Sara Bortolani Mauro Monforte Enzo Ricci Fiorella Piemonte Giorgio Tasca Redox Homeostasis in Muscular Dystrophies Cells muscular dystrophies FSHD oxidative stress reactive oxygen species (ROS) antioxidants Nrf2 |
| title | Redox Homeostasis in Muscular Dystrophies |
| title_full | Redox Homeostasis in Muscular Dystrophies |
| title_fullStr | Redox Homeostasis in Muscular Dystrophies |
| title_full_unstemmed | Redox Homeostasis in Muscular Dystrophies |
| title_short | Redox Homeostasis in Muscular Dystrophies |
| title_sort | redox homeostasis in muscular dystrophies |
| topic | muscular dystrophies FSHD oxidative stress reactive oxygen species (ROS) antioxidants Nrf2 |
| url | https://www.mdpi.com/2073-4409/10/6/1364 |
| work_keys_str_mv | AT nicolamosca redoxhomeostasisinmusculardystrophies AT sarapetrillo redoxhomeostasisinmusculardystrophies AT sarabortolani redoxhomeostasisinmusculardystrophies AT mauromonforte redoxhomeostasisinmusculardystrophies AT enzoricci redoxhomeostasisinmusculardystrophies AT fiorellapiemonte redoxhomeostasisinmusculardystrophies AT giorgiotasca redoxhomeostasisinmusculardystrophies |