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