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Skeletal Muscle-Specific Ablation of γ(cyto)-Actin Does Not Exacerbate the mdx Phenotype

We previously documented a ten-fold increase in γ(cyto)-actin expression in dystrophin-deficient skeletal muscle and hypothesized that increased γ(cyto)-actin expression may participate in an adaptive cytoskeletal remodeling response. To explore whether increased γ(cyto)-actin fortifies the cortical...

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Detalles Bibliográficos
Autores principales: Prins, Kurt W., Lowe, Dawn A., Ervasti, James M.
Formato: Texto
Lenguaje:English
Publicado: Public Library of Science 2008
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2409075/
https://www.ncbi.nlm.nih.gov/pubmed/18545671
http://dx.doi.org/10.1371/journal.pone.0002419
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author Prins, Kurt W.
Lowe, Dawn A.
Ervasti, James M.
author_facet Prins, Kurt W.
Lowe, Dawn A.
Ervasti, James M.
author_sort Prins, Kurt W.
collection PubMed
description We previously documented a ten-fold increase in γ(cyto)-actin expression in dystrophin-deficient skeletal muscle and hypothesized that increased γ(cyto)-actin expression may participate in an adaptive cytoskeletal remodeling response. To explore whether increased γ(cyto)-actin fortifies the cortical cytoskeleton in dystrophic skeletal muscle, we generated double knockout mice lacking both dystrophin and γ(cyto)-actin specifically in skeletal muscle (ms-DKO). Surprisingly, dystrophin-deficient mdx and ms-DKO mice presented with comparable levels of myofiber necrosis, membrane instability, and deficits in muscle function. The lack of an exacerbated phenotype in ms-DKO mice suggests γ(cyto)-actin and dystrophin function in a common pathway. Finally, because both mdx and ms-DKO skeletal muscle showed similar levels of utrophin expression and presented with identical dystrophies, we conclude utrophin can partially compensate for the loss of dystrophin independent of a γ(cyto)-actin-utrophin interaction.
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spelling pubmed-24090752008-06-11 Skeletal Muscle-Specific Ablation of γ(cyto)-Actin Does Not Exacerbate the mdx Phenotype Prins, Kurt W. Lowe, Dawn A. Ervasti, James M. PLoS One Research Article We previously documented a ten-fold increase in γ(cyto)-actin expression in dystrophin-deficient skeletal muscle and hypothesized that increased γ(cyto)-actin expression may participate in an adaptive cytoskeletal remodeling response. To explore whether increased γ(cyto)-actin fortifies the cortical cytoskeleton in dystrophic skeletal muscle, we generated double knockout mice lacking both dystrophin and γ(cyto)-actin specifically in skeletal muscle (ms-DKO). Surprisingly, dystrophin-deficient mdx and ms-DKO mice presented with comparable levels of myofiber necrosis, membrane instability, and deficits in muscle function. The lack of an exacerbated phenotype in ms-DKO mice suggests γ(cyto)-actin and dystrophin function in a common pathway. Finally, because both mdx and ms-DKO skeletal muscle showed similar levels of utrophin expression and presented with identical dystrophies, we conclude utrophin can partially compensate for the loss of dystrophin independent of a γ(cyto)-actin-utrophin interaction. Public Library of Science 2008-06-11 /pmc/articles/PMC2409075/ /pubmed/18545671 http://dx.doi.org/10.1371/journal.pone.0002419 Text en Prins et al. http://creativecommons.org/licenses/by/4.0/ This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are properly credited.
spellingShingle Research Article
Prins, Kurt W.
Lowe, Dawn A.
Ervasti, James M.
Skeletal Muscle-Specific Ablation of γ(cyto)-Actin Does Not Exacerbate the mdx Phenotype
title Skeletal Muscle-Specific Ablation of γ(cyto)-Actin Does Not Exacerbate the mdx Phenotype
title_full Skeletal Muscle-Specific Ablation of γ(cyto)-Actin Does Not Exacerbate the mdx Phenotype
title_fullStr Skeletal Muscle-Specific Ablation of γ(cyto)-Actin Does Not Exacerbate the mdx Phenotype
title_full_unstemmed Skeletal Muscle-Specific Ablation of γ(cyto)-Actin Does Not Exacerbate the mdx Phenotype
title_short Skeletal Muscle-Specific Ablation of γ(cyto)-Actin Does Not Exacerbate the mdx Phenotype
title_sort skeletal muscle-specific ablation of γ(cyto)-actin does not exacerbate the mdx phenotype
topic Research Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2409075/
https://www.ncbi.nlm.nih.gov/pubmed/18545671
http://dx.doi.org/10.1371/journal.pone.0002419
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