β1-syntrophin modulation by miR-222 in mdx mice.

<h4>Background</h4>In mdx mice, the absence of dystrophin leads to the deficiency of other components of the dystrophin-glycoprotein complex (DAPC), making skeletal muscle fibers more susceptible to necrosis. The mechanisms involved in the disappearance of the DAPC are not completely und...

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Autores principales:	Valeria De Arcangelis, Filippo Serra, Carlo Cogoni, Elisabetta Vivarelli, Lucia Monaco, Fabio Naro
Formato:	article
Lenguaje:	EN
Publicado:	Public Library of Science (PLoS) 2010
Materias:	Medicine R Science Q
Acceso en línea:	https://doaj.org/article/6f01955e5e9d4d18b70f238d32ac5180
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spelling	oai:doaj.org-article:6f01955e5e9d4d18b70f238d32ac51802021-11-18T06:36:12Zβ1-syntrophin modulation by miR-222 in mdx mice.1932-620310.1371/journal.pone.0012098https://doaj.org/article/6f01955e5e9d4d18b70f238d32ac51802010-08-01T00:00:00Zhttps://www.ncbi.nlm.nih.gov/pmc/articles/pmid/20856896/pdf/?tool=EBIhttps://doaj.org/toc/1932-6203<h4>Background</h4>In mdx mice, the absence of dystrophin leads to the deficiency of other components of the dystrophin-glycoprotein complex (DAPC), making skeletal muscle fibers more susceptible to necrosis. The mechanisms involved in the disappearance of the DAPC are not completely understood. The muscles of mdx mice express normal amounts of mRNA for the DAPC components, thus suggesting post-transcriptional regulation.<h4>Methodology/principal findings</h4>We investigated the hypothesis that DAPC reduction could be associated with the microRNA system. Among the possible microRNAs (miRs) found to be upregulated in the skeletal muscle tissue of mdx compared to wt mice, we demonstrated that miR-222 specifically binds to the 3'-UTR of β1-syntrophin and participates in the downregulation of β1-syntrophin. In addition, we documented an altered regulation of the 3'-UTR of β1-syntrophin in muscle tissue from dystrophic mice.<h4>Conclusion/significance</h4>These results show the importance of the microRNA system in the regulation of DAPC components in dystrophic muscle, and suggest a potential role of miRs in the pathophysiology of dystrophy.Valeria De ArcangelisFilippo SerraCarlo CogoniElisabetta VivarelliLucia MonacoFabio NaroPublic Library of Science (PLoS)articleMedicineRScienceQENPLoS ONE, Vol 5, Iss 8 (2010)
institution	DOAJ
collection	DOAJ
language	EN
topic	Medicine R Science Q
spellingShingle	Medicine R Science Q Valeria De Arcangelis Filippo Serra Carlo Cogoni Elisabetta Vivarelli Lucia Monaco Fabio Naro β1-syntrophin modulation by miR-222 in mdx mice.
description	<h4>Background</h4>In mdx mice, the absence of dystrophin leads to the deficiency of other components of the dystrophin-glycoprotein complex (DAPC), making skeletal muscle fibers more susceptible to necrosis. The mechanisms involved in the disappearance of the DAPC are not completely understood. The muscles of mdx mice express normal amounts of mRNA for the DAPC components, thus suggesting post-transcriptional regulation.<h4>Methodology/principal findings</h4>We investigated the hypothesis that DAPC reduction could be associated with the microRNA system. Among the possible microRNAs (miRs) found to be upregulated in the skeletal muscle tissue of mdx compared to wt mice, we demonstrated that miR-222 specifically binds to the 3'-UTR of β1-syntrophin and participates in the downregulation of β1-syntrophin. In addition, we documented an altered regulation of the 3'-UTR of β1-syntrophin in muscle tissue from dystrophic mice.<h4>Conclusion/significance</h4>These results show the importance of the microRNA system in the regulation of DAPC components in dystrophic muscle, and suggest a potential role of miRs in the pathophysiology of dystrophy.
format	article
author	Valeria De Arcangelis Filippo Serra Carlo Cogoni Elisabetta Vivarelli Lucia Monaco Fabio Naro
author_facet	Valeria De Arcangelis Filippo Serra Carlo Cogoni Elisabetta Vivarelli Lucia Monaco Fabio Naro
author_sort	Valeria De Arcangelis
title	β1-syntrophin modulation by miR-222 in mdx mice.
title_short	β1-syntrophin modulation by miR-222 in mdx mice.
title_full	β1-syntrophin modulation by miR-222 in mdx mice.
title_fullStr	β1-syntrophin modulation by miR-222 in mdx mice.
title_full_unstemmed	β1-syntrophin modulation by miR-222 in mdx mice.
title_sort	β1-syntrophin modulation by mir-222 in mdx mice.
publisher	Public Library of Science (PLoS)
publishDate	2010
url	https://doaj.org/article/6f01955e5e9d4d18b70f238d32ac5180
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_version_	1718424421228609536

β1-syntrophin modulation by miR-222 in mdx mice.

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