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

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.

Bibliographic Details
Main Authors: Marcela P. Cataldi, Peijuan Lu, Anthony Blaeser, Qi Long Lu
Format: Article
Language:English
Published: Nature Publishing Group 2018-08-01
Series:Nature Communications
Online Access:https://doi.org/10.1038/s41467-018-05990-z
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spelling doaj-88d48ec703f1413292d3648e7e85ca532021-05-11T09:39:06ZengNature Publishing GroupNature Communications2041-17232018-08-019111210.1038/s41467-018-05990-zRibitol restores functionally glycosylated α-dystroglycan and improves muscle function in dystrophic FKRP-mutant miceMarcela P. Cataldi0Peijuan Lu1Anthony Blaeser2Qi Long Lu3McColl-Lockwood Laboratory for Muscular Dystrophy Research, Cannon Research Center, Carolinas Medical Center, Carolinas Healthcare SystemMcColl-Lockwood Laboratory for Muscular Dystrophy Research, Cannon Research Center, Carolinas Medical Center, Carolinas Healthcare SystemMcColl-Lockwood Laboratory for Muscular Dystrophy Research, Cannon Research Center, Carolinas Medical Center, Carolinas Healthcare SystemMcColl-Lockwood Laboratory for Muscular Dystrophy Research, Cannon Research Center, Carolinas Medical Center, Carolinas Healthcare SystemMutations 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.https://doi.org/10.1038/s41467-018-05990-z
collection DOAJ
language English
format Article
sources DOAJ
author Marcela P. Cataldi
Peijuan Lu
Anthony Blaeser
Qi Long Lu
spellingShingle Marcela P. Cataldi
Peijuan Lu
Anthony Blaeser
Qi Long Lu
Ribitol restores functionally glycosylated α-dystroglycan and improves muscle function in dystrophic FKRP-mutant mice
Nature Communications
author_facet Marcela P. Cataldi
Peijuan Lu
Anthony Blaeser
Qi Long Lu
author_sort Marcela P. Cataldi
title Ribitol restores functionally glycosylated α-dystroglycan and improves muscle function in dystrophic FKRP-mutant mice
title_short Ribitol restores functionally glycosylated α-dystroglycan and improves muscle function in dystrophic FKRP-mutant mice
title_full Ribitol restores functionally glycosylated α-dystroglycan and improves muscle function in dystrophic FKRP-mutant mice
title_fullStr Ribitol restores functionally glycosylated α-dystroglycan and improves muscle function in dystrophic FKRP-mutant mice
title_full_unstemmed Ribitol restores functionally glycosylated α-dystroglycan and improves muscle function in dystrophic FKRP-mutant mice
title_sort ribitol restores functionally glycosylated α-dystroglycan and improves muscle function in dystrophic fkrp-mutant mice
publisher Nature Publishing Group
series Nature Communications
issn 2041-1723
publishDate 2018-08-01
description 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.
url https://doi.org/10.1038/s41467-018-05990-z
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AT peijuanlu ribitolrestoresfunctionallyglycosylatedadystroglycanandimprovesmusclefunctionindystrophicfkrpmutantmice
AT anthonyblaeser ribitolrestoresfunctionallyglycosylatedadystroglycanandimprovesmusclefunctionindystrophicfkrpmutantmice
AT qilonglu ribitolrestoresfunctionallyglycosylatedadystroglycanandimprovesmusclefunctionindystrophicfkrpmutantmice
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