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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...

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Detalles Bibliográficos
Autores principales: Michelucci, Antonio, De Marco, Alessandro, Guarnier, Flavia A., Protasi, Feliciano, Boncompagni, Simona
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Hindawi 2017
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