Antioxidant Treatment Reduces Formation of Structural Cores and Improves Muscle Function in RYR1Y522SWT Mice
Joint Authors
Protasi, Feliciano
Boncompagni, Simona
Michelucci, Antonio
De Marco, Alessandro
Guarnier, Flavia A.
Source
Oxidative Medicine and Cellular Longevity
Issue
Vol. 2017, Issue 2017 (31 Dec. 2017), pp.1-15, 15 p.
Publisher
Hindawi Publishing Corporation
Publication Date
2017-09-10
Country of Publication
Egypt
No. of Pages
15
Main Subjects
Abstract EN
Central core disease (CCD) is a congenital myopathy linked to mutations in the ryanodine receptor type 1 (RYR1), the sarcoplasmic reticulum Ca2+ release channel of skeletal muscle.
CCD is characterized by formation of amorphous cores within muscle fibers, lacking mitochondrial activity.
In skeletal muscle of RYR1Y522S/WT knock-in mice, carrying a human mutation in RYR1 linked to malignant hyperthermia (MH) with cores, oxidative stress is elevated and fibers present severe mitochondrial damage and cores.
We treated RYR1Y522S/WT mice with N-acetylcysteine (NAC), an antioxidant provided ad libitum in drinking water for either 2 or 6 months.
Our results show that 2 months of NAC treatment starting at 2 months of age, when mitochondrial and fiber damage was still minimal, (i) reduce formation of unstructured and contracture cores, (ii) improve muscle function, and (iii) decrease mitochondrial damage.
The beneficial effect of NAC treatment is also evident following 6 months of treatment starting at 4 months of age, when structural damage was at an advanced stage.
NAC exerts its protective effect likely by lowering oxidative stress, as supported by the reduction of 3-NT and SOD2 levels.
This work suggests that NAC administration is beneficial to prevent mitochondrial damage and formation of cores and improve muscle function in RYR1Y522S/WT mice.
American Psychological Association (APA)
Michelucci, Antonio& De Marco, Alessandro& Guarnier, Flavia A.& Protasi, Feliciano& Boncompagni, Simona. 2017. Antioxidant Treatment Reduces Formation of Structural Cores and Improves Muscle Function in RYR1Y522SWT Mice. Oxidative Medicine and Cellular Longevity،Vol. 2017, no. 2017, pp.1-15.
https://search.emarefa.net/detail/BIM-1195306
Modern Language Association (MLA)
Michelucci, Antonio…[et al.]. Antioxidant Treatment Reduces Formation of Structural Cores and Improves Muscle Function in RYR1Y522SWT Mice. Oxidative Medicine and Cellular Longevity No. 2017 (2017), pp.1-15.
https://search.emarefa.net/detail/BIM-1195306
American Medical Association (AMA)
Michelucci, Antonio& De Marco, Alessandro& Guarnier, Flavia A.& Protasi, Feliciano& Boncompagni, Simona. Antioxidant Treatment Reduces Formation of Structural Cores and Improves Muscle Function in RYR1Y522SWT Mice. Oxidative Medicine and Cellular Longevity. 2017. Vol. 2017, no. 2017, pp.1-15.
https://search.emarefa.net/detail/BIM-1195306
Data Type
Journal Articles
Language
English
Notes
Includes bibliographical references
Record ID
BIM-1195306