Ribitol dose-dependently enhances matriglycan expression and improves muscle function with prolonged life span in limb girdle muscular dystrophy 2I mouse model

Limb Girdle Muscular Dystrophy 2I (LGMDR9) is one of the most common LGMD characterized by defects in glycosylation of α-dystroglycan (matriglycan) resulting from mutations of Fukutin-related protein (FKRP). There is no effective therapy currently available. We recently demonstrated that ribitol sup...

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Detalles Bibliográficos
Autores principales: Wu, Bo, Drains, Morgan, Shah, Sapana N., Lu, Pei Juan, Leroy, Victoria, Killilee, Jessalyn, Rawls, Raegan, Tucker, Jason D., Blaeser, Anthony, Lu, Qi Long
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Public Library of Science 2022
Materias:
Research Article
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9714851/
https://www.ncbi.nlm.nih.gov/pubmed/36454905
http://dx.doi.org/10.1371/journal.pone.0278482

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