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Antioxidant Treatment Reduces Formation of Structural Cores and Improves Muscle Function in RYR1(Y522S/WT) Mice
Central core disease (CCD) is a congenital myopathy linked to mutations in the ryanodine receptor type 1 (RYR1), the sarcoplasmic reticulum Ca(2+) release channel of skeletal muscle. CCD is characterized by formation of amorphous cores within muscle fibers, lacking mitochondrial activity. In skeleta...
Autores principales: | Michelucci, Antonio, De Marco, Alessandro, Guarnier, Flavia A., Protasi, Feliciano, Boncompagni, Simona |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Hindawi
2017
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5610828/ https://www.ncbi.nlm.nih.gov/pubmed/29062463 http://dx.doi.org/10.1155/2017/6792694 |
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