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.
Main Authors: | , , , |
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Format: | Article |
Language: | English |
Published: |
Nature Publishing Group
2018-08-01
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Series: | Nature Communications |
Online Access: | https://doi.org/10.1038/s41467-018-05990-z |