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Emery–Dreifuss muscular dystrophy–linked genes and centronuclear myopathy–linked genes regulate myonuclear movement by distinct mechanisms
Muscle cells are a syncytium in which the many nuclei are positioned to maximize the distance between adjacent nuclei. Although mispositioned nuclei are correlated with many muscle disorders, it is not known whether this common phenotype is the result of a common mechanism. To answer this question,...
Autores principales: | , , , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
The American Society for Cell Biology
2017
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5555658/ https://www.ncbi.nlm.nih.gov/pubmed/28637766 http://dx.doi.org/10.1091/mbc.E16-10-0721 |
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author | Collins, Mary Ann Mandigo, Torrey R. Camuglia, Jaclyn M. Vazquez, Gabriella A. Anderson, Alyssa J. Hudson, Christine H. Hanron, John L. Folker, Eric S. |
author_facet | Collins, Mary Ann Mandigo, Torrey R. Camuglia, Jaclyn M. Vazquez, Gabriella A. Anderson, Alyssa J. Hudson, Christine H. Hanron, John L. Folker, Eric S. |
author_sort | Collins, Mary Ann |
collection | PubMed |
description | Muscle cells are a syncytium in which the many nuclei are positioned to maximize the distance between adjacent nuclei. Although mispositioned nuclei are correlated with many muscle disorders, it is not known whether this common phenotype is the result of a common mechanism. To answer this question, we disrupted the expression of genes linked to Emery–Dreifuss muscular dystrophy (EDMD) and centronuclear myopathy (CNM) in Drosophila and evaluated the position of the nuclei. We found that the genes linked to EDMD and CNM were each necessary to properly position nuclei. However, the specific phenotypes were different. EDMD-linked genes were necessary for the initial separation of nuclei into distinct clusters, suggesting that these factors relieve interactions between nuclei. CNM-linked genes were necessary to maintain the nuclei within clusters as they moved toward the muscle ends, suggesting that these factors were necessary to maintain interactions between nuclei. Together these data suggest that nuclear position is disrupted by distinct mechanisms in EDMD and CNM. |
format | Online Article Text |
id | pubmed-5555658 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2017 |
publisher | The American Society for Cell Biology |
record_format | MEDLINE/PubMed |
spelling | pubmed-55556582017-10-30 Emery–Dreifuss muscular dystrophy–linked genes and centronuclear myopathy–linked genes regulate myonuclear movement by distinct mechanisms Collins, Mary Ann Mandigo, Torrey R. Camuglia, Jaclyn M. Vazquez, Gabriella A. Anderson, Alyssa J. Hudson, Christine H. Hanron, John L. Folker, Eric S. Mol Biol Cell Articles Muscle cells are a syncytium in which the many nuclei are positioned to maximize the distance between adjacent nuclei. Although mispositioned nuclei are correlated with many muscle disorders, it is not known whether this common phenotype is the result of a common mechanism. To answer this question, we disrupted the expression of genes linked to Emery–Dreifuss muscular dystrophy (EDMD) and centronuclear myopathy (CNM) in Drosophila and evaluated the position of the nuclei. We found that the genes linked to EDMD and CNM were each necessary to properly position nuclei. However, the specific phenotypes were different. EDMD-linked genes were necessary for the initial separation of nuclei into distinct clusters, suggesting that these factors relieve interactions between nuclei. CNM-linked genes were necessary to maintain the nuclei within clusters as they moved toward the muscle ends, suggesting that these factors were necessary to maintain interactions between nuclei. Together these data suggest that nuclear position is disrupted by distinct mechanisms in EDMD and CNM. The American Society for Cell Biology 2017-08-15 /pmc/articles/PMC5555658/ /pubmed/28637766 http://dx.doi.org/10.1091/mbc.E16-10-0721 Text en © 2017 Collins, Mandigo, et al. This article is distributed by The American Society for Cell Biology under license from the author(s). Two months after publication it is available to the public under an Attribution–Noncommercial–Share Alike 3.0 Unported Creative Commons License (http://creativecommons.org/licenses/by-nc-sa/3.0). “ASCB®,” “The American Society for Cell Biology®,” and “Molecular Biology of the Cell®” are registered trademarks of The American Society for Cell Biology. |
spellingShingle | Articles Collins, Mary Ann Mandigo, Torrey R. Camuglia, Jaclyn M. Vazquez, Gabriella A. Anderson, Alyssa J. Hudson, Christine H. Hanron, John L. Folker, Eric S. Emery–Dreifuss muscular dystrophy–linked genes and centronuclear myopathy–linked genes regulate myonuclear movement by distinct mechanisms |
title | Emery–Dreifuss muscular dystrophy–linked genes and centronuclear myopathy–linked genes regulate myonuclear movement by distinct mechanisms |
title_full | Emery–Dreifuss muscular dystrophy–linked genes and centronuclear myopathy–linked genes regulate myonuclear movement by distinct mechanisms |
title_fullStr | Emery–Dreifuss muscular dystrophy–linked genes and centronuclear myopathy–linked genes regulate myonuclear movement by distinct mechanisms |
title_full_unstemmed | Emery–Dreifuss muscular dystrophy–linked genes and centronuclear myopathy–linked genes regulate myonuclear movement by distinct mechanisms |
title_short | Emery–Dreifuss muscular dystrophy–linked genes and centronuclear myopathy–linked genes regulate myonuclear movement by distinct mechanisms |
title_sort | emery–dreifuss muscular dystrophy–linked genes and centronuclear myopathy–linked genes regulate myonuclear movement by distinct mechanisms |
topic | Articles |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5555658/ https://www.ncbi.nlm.nih.gov/pubmed/28637766 http://dx.doi.org/10.1091/mbc.E16-10-0721 |
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