Ribitol restores functionally glycosylated α-dystroglycan and improves muscle function in dystrophic FKRP-mutant mice

Mutations in FKRP impair glycosylation of alpha-dystroglycan, leading to muscular dystrophy. Here, the authors show that oral administration of ribitol increases dystropglycan glycosylation and ameliorates symptoms of muscular dystrophy in FKRP-deficient mouse models.

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Detalles Bibliográficos
Autores principales: Marcela P. Cataldi, Peijuan Lu, Anthony Blaeser, Qi Long Lu
Formato: article
Lenguaje:EN
Publicado: Nature Portfolio 2018
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Q
Acceso en línea:https://doaj.org/article/88d48ec703f1413292d3648e7e85ca53
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