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
Formato:	article
Lenguaje:	EN
Publicado:	Nature Portfolio 2018
Materias:	Science Q
Acceso en línea:	https://doaj.org/article/88d48ec703f1413292d3648e7e85ca53
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spelling	oai:doaj.org-article:88d48ec703f1413292d3648e7e85ca532021-12-02T14:41:17ZRibitol restores functionally glycosylated α-dystroglycan and improves muscle function in dystrophic FKRP-mutant mice10.1038/s41467-018-05990-z2041-1723https://doaj.org/article/88d48ec703f1413292d3648e7e85ca532018-08-01T00:00:00Zhttps://doi.org/10.1038/s41467-018-05990-zhttps://doaj.org/toc/2041-1723Mutations 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.Marcela P. CataldiPeijuan LuAnthony BlaeserQi Long LuNature PortfolioarticleScienceQENNature Communications, Vol 9, Iss 1, Pp 1-12 (2018)
institution	DOAJ
collection	DOAJ
language	EN
topic	Science Q
spellingShingle	Science Q 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
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.
format	article
author	Marcela P. Cataldi Peijuan Lu Anthony Blaeser Qi Long Lu
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 Portfolio
publishDate	2018
url	https://doaj.org/article/88d48ec703f1413292d3648e7e85ca53
work_keys_str_mv	AT marcelapcataldi ribitolrestoresfunctionallyglycosylatedadystroglycanandimprovesmusclefunctionindystrophicfkrpmutantmice AT peijuanlu ribitolrestoresfunctionallyglycosylatedadystroglycanandimprovesmusclefunctionindystrophicfkrpmutantmice AT anthonyblaeser ribitolrestoresfunctionallyglycosylatedadystroglycanandimprovesmusclefunctionindystrophicfkrpmutantmice AT qilonglu ribitolrestoresfunctionallyglycosylatedadystroglycanandimprovesmusclefunctionindystrophicfkrpmutantmice
_version_	1718389941652684800

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

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