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

Authors :
Marcela P. Cataldi
Peijuan Lu
Anthony Blaeser
Qi Long Lu
Source :
Nature Communications, Vol 9, Iss 1, Pp 1-12 (2018)
Publication Year :
2018
Publisher :
Nature Portfolio, 2018.

Abstract

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.

Subjects

Subjects :
Science

Details

Language :
English
ISSN :
20411723
Volume :
9
Issue :
1
Database :
Directory of Open Access Journals
Journal :
Nature Communications
Publication Type :
Academic Journal
Accession number :
edsdoj.88d48ec703f1413292d3648e7e85ca53
Document Type :
article
Full Text :
https://doi.org/10.1038/s41467-018-05990-z