Revertant fibers in the mdx murine model of Duchenne muscular dystrophy: an age- and muscle-related reappraisal.

Muscles in Duchenne dystrophy patients are characterized by the absence of dystrophin, yet transverse sections show a small percentage of fibers (termed "revertant fibers") positive for dystrophin expression. This phenomenon, whose biological bases have not been fully elucidated, is presen...

Descripción completa

Guardado en:

Detalles Bibliográficos
Autores principales:	Sarah R Pigozzo, Lorena Da Re, Chiara Romualdi, Pietro G Mazzara, Eva Galletta, Sue Fletcher, Stephen D Wilton, Libero Vitiello
Formato:	article
Lenguaje:	EN
Publicado:	Public Library of Science (PLoS) 2013
Materias:	Medicine R Science Q
Acceso en línea:	https://doaj.org/article/2061e3b8fd84469c8003ff7b5d3d1b59
Etiquetas:	Agregar Etiqueta Sin Etiquetas, Sea el primero en etiquetar este registro!

id	oai:doaj.org-article:2061e3b8fd84469c8003ff7b5d3d1b59
record_format	dspace
spelling	oai:doaj.org-article:2061e3b8fd84469c8003ff7b5d3d1b592021-11-18T08:57:55ZRevertant fibers in the mdx murine model of Duchenne muscular dystrophy: an age- and muscle-related reappraisal.1932-620310.1371/journal.pone.0072147https://doaj.org/article/2061e3b8fd84469c8003ff7b5d3d1b592013-01-01T00:00:00Zhttps://www.ncbi.nlm.nih.gov/pmc/articles/pmid/24015212/pdf/?tool=EBIhttps://doaj.org/toc/1932-6203Muscles in Duchenne dystrophy patients are characterized by the absence of dystrophin, yet transverse sections show a small percentage of fibers (termed "revertant fibers") positive for dystrophin expression. This phenomenon, whose biological bases have not been fully elucidated, is present also in the murine and canine models of DMD and can confound the evaluation of therapeutic approaches. We analyzed 11 different muscles in a cohort of 40 mdx mice, the most commonly model used in pre-clinical studies, belonging to four age groups; such number of animals allowed us to perform solid ANOVA statistical analysis. We assessed the average number of dystrophin-positive fibers, both absolute and normalized for muscle size, and the correlation between their formation and the ageing process. Our results indicate that various muscles develop different numbers of revertant fibers, with different time trends; besides, they suggest that the biological mechanism(s) behind dystrophin re-expression might not be limited to the early development phases but could actually continue during adulthood. Importantly, such finding was seen also in cardiac muscle, a fact that does not fit into the current hypothesis of the clonal origin of "revertant" myonuclei from satellite cells. This work represents the largest, statistically significant analysis of revertant fibers in mdx mice so far, which can now be used as a reference point for improving the evaluation of therapeutic approaches for DMD. At the same time, it provides new clues about the formation of revertant fibers/cardiomyocytes in dystrophic skeletal and cardiac muscle.Sarah R PigozzoLorena Da ReChiara RomualdiPietro G MazzaraEva GallettaSue FletcherStephen D WiltonLibero VitielloPublic Library of Science (PLoS)articleMedicineRScienceQENPLoS ONE, Vol 8, Iss 8, p e72147 (2013)
institution	DOAJ
collection	DOAJ
language	EN
topic	Medicine R Science Q
spellingShingle	Medicine R Science Q Sarah R Pigozzo Lorena Da Re Chiara Romualdi Pietro G Mazzara Eva Galletta Sue Fletcher Stephen D Wilton Libero Vitiello Revertant fibers in the mdx murine model of Duchenne muscular dystrophy: an age- and muscle-related reappraisal.
description	Muscles in Duchenne dystrophy patients are characterized by the absence of dystrophin, yet transverse sections show a small percentage of fibers (termed "revertant fibers") positive for dystrophin expression. This phenomenon, whose biological bases have not been fully elucidated, is present also in the murine and canine models of DMD and can confound the evaluation of therapeutic approaches. We analyzed 11 different muscles in a cohort of 40 mdx mice, the most commonly model used in pre-clinical studies, belonging to four age groups; such number of animals allowed us to perform solid ANOVA statistical analysis. We assessed the average number of dystrophin-positive fibers, both absolute and normalized for muscle size, and the correlation between their formation and the ageing process. Our results indicate that various muscles develop different numbers of revertant fibers, with different time trends; besides, they suggest that the biological mechanism(s) behind dystrophin re-expression might not be limited to the early development phases but could actually continue during adulthood. Importantly, such finding was seen also in cardiac muscle, a fact that does not fit into the current hypothesis of the clonal origin of "revertant" myonuclei from satellite cells. This work represents the largest, statistically significant analysis of revertant fibers in mdx mice so far, which can now be used as a reference point for improving the evaluation of therapeutic approaches for DMD. At the same time, it provides new clues about the formation of revertant fibers/cardiomyocytes in dystrophic skeletal and cardiac muscle.
format	article
author	Sarah R Pigozzo Lorena Da Re Chiara Romualdi Pietro G Mazzara Eva Galletta Sue Fletcher Stephen D Wilton Libero Vitiello
author_facet	Sarah R Pigozzo Lorena Da Re Chiara Romualdi Pietro G Mazzara Eva Galletta Sue Fletcher Stephen D Wilton Libero Vitiello
author_sort	Sarah R Pigozzo
title	Revertant fibers in the mdx murine model of Duchenne muscular dystrophy: an age- and muscle-related reappraisal.
title_short	Revertant fibers in the mdx murine model of Duchenne muscular dystrophy: an age- and muscle-related reappraisal.
title_full	Revertant fibers in the mdx murine model of Duchenne muscular dystrophy: an age- and muscle-related reappraisal.
title_fullStr	Revertant fibers in the mdx murine model of Duchenne muscular dystrophy: an age- and muscle-related reappraisal.
title_full_unstemmed	Revertant fibers in the mdx murine model of Duchenne muscular dystrophy: an age- and muscle-related reappraisal.
title_sort	revertant fibers in the mdx murine model of duchenne muscular dystrophy: an age- and muscle-related reappraisal.
publisher	Public Library of Science (PLoS)
publishDate	2013
url	https://doaj.org/article/2061e3b8fd84469c8003ff7b5d3d1b59
work_keys_str_mv	AT sarahrpigozzo revertantfibersinthemdxmurinemodelofduchennemusculardystrophyanageandmusclerelatedreappraisal AT lorenadare revertantfibersinthemdxmurinemodelofduchennemusculardystrophyanageandmusclerelatedreappraisal AT chiararomualdi revertantfibersinthemdxmurinemodelofduchennemusculardystrophyanageandmusclerelatedreappraisal AT pietrogmazzara revertantfibersinthemdxmurinemodelofduchennemusculardystrophyanageandmusclerelatedreappraisal AT evagalletta revertantfibersinthemdxmurinemodelofduchennemusculardystrophyanageandmusclerelatedreappraisal AT suefletcher revertantfibersinthemdxmurinemodelofduchennemusculardystrophyanageandmusclerelatedreappraisal AT stephendwilton revertantfibersinthemdxmurinemodelofduchennemusculardystrophyanageandmusclerelatedreappraisal AT liberovitiello revertantfibersinthemdxmurinemodelofduchennemusculardystrophyanageandmusclerelatedreappraisal
_version_	1718421068283117568

Revertant fibers in the mdx murine model of Duchenne muscular dystrophy: an age- and muscle-related reappraisal.

Ejemplares similares