A chemical chaperone improves muscle function in mice with a RyR1 mutation

A chemical chaperone improves muscle function in mice with a RyR1 mutation

Mutations in the RyR1 channel cause core myopathies. Here the authors show that ER stress and the unfolded protein response underlie the pathology caused by a common RyR1 channel mutation, and show that treatment with a chemical chaperone restores muscle function in mice.

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Detalles Bibliográficos
Autores principales: Chang Seok Lee, Amy D. Hanna, Hui Wang, Adan Dagnino-Acosta, Aditya D. Joshi, Mark Knoblauch, Yan Xia, Dimitra K. Georgiou, Jianjun Xu, Cheng Long, Hisayuki Amano, Corey Reynolds, Keke Dong, John C. Martin, William R. Lagor, George G. Rodney, Ergun Sahin, Caroline Sewry, Susan L. Hamilton
Formato: article
Lenguaje:EN
Publicado: Nature Portfolio 2017
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Science
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Acceso en línea:https://doaj.org/article/8a7faf752203416684267602ebe0758b
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