Therapeutic potential of matrix metalloproteinases in Duchenne muscular dystrophy
Matrix metalloproteinases (MMPs) are secreted proteinases that have physiologic roles in degradation and remodeling of extracellular matrix (ECM) in almost all tissues. However, their excessive production in disease conditions leads to many pathological features including tissue breakdown, inflammat...
Autores principales: | , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
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Frontiers Media S.A.
2014
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Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4207008/ https://www.ncbi.nlm.nih.gov/pubmed/25364719 http://dx.doi.org/10.3389/fcell.2014.00011 |
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author | Ogura, Yuji Tajrishi, Marjan M. Sato, Shuichi Hindi, Sajedah M. Kumar, Ashok |
author_facet | Ogura, Yuji Tajrishi, Marjan M. Sato, Shuichi Hindi, Sajedah M. Kumar, Ashok |
author_sort | Ogura, Yuji |
collection | PubMed |
description | Matrix metalloproteinases (MMPs) are secreted proteinases that have physiologic roles in degradation and remodeling of extracellular matrix (ECM) in almost all tissues. However, their excessive production in disease conditions leads to many pathological features including tissue breakdown, inflammation, cell death, and fibrosis. Duchenne Muscular dystrophy (DMD) is a devastating genetic muscle disorder caused by partial or complete loss of cytoskeletal protein dystrophin. Progressive muscle wasting in DMD is accompanied by myofiber necrosis followed by cycles of regeneration and degeneration and inflammation that eventually result in replacement of myofiber by connective and adipose tissues. Emerging evidence suggests that gene expression and the activity of various MMPs are aberrantly regulated in muscle biopsies from DMD patients and in skeletal muscle of animal models of DMD. Moreover, a few studies employing genetic mouse models have revealed that different MMPs play distinct roles in disease progression in DMD. Modulation of the activity of MMPs improves myofiber regeneration and enhances the efficacy of transplantation and engraftment of muscle progenitor cells in dystrophic muscle in mouse models of DMD. Furthermore, recent reports also suggest that some MMPs especially MMP-9 can serve as a biomarker for diagnosis and prognosis of DMD. In this article, we provide a succinct overview of the regulation of various MMPs and their therapeutic importance in DMD. |
format | Online Article Text |
id | pubmed-4207008 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2014 |
publisher | Frontiers Media S.A. |
record_format | MEDLINE/PubMed |
spelling | pubmed-42070082014-10-31 Therapeutic potential of matrix metalloproteinases in Duchenne muscular dystrophy Ogura, Yuji Tajrishi, Marjan M. Sato, Shuichi Hindi, Sajedah M. Kumar, Ashok Front Cell Dev Biol Cell and Developmental Biology Matrix metalloproteinases (MMPs) are secreted proteinases that have physiologic roles in degradation and remodeling of extracellular matrix (ECM) in almost all tissues. However, their excessive production in disease conditions leads to many pathological features including tissue breakdown, inflammation, cell death, and fibrosis. Duchenne Muscular dystrophy (DMD) is a devastating genetic muscle disorder caused by partial or complete loss of cytoskeletal protein dystrophin. Progressive muscle wasting in DMD is accompanied by myofiber necrosis followed by cycles of regeneration and degeneration and inflammation that eventually result in replacement of myofiber by connective and adipose tissues. Emerging evidence suggests that gene expression and the activity of various MMPs are aberrantly regulated in muscle biopsies from DMD patients and in skeletal muscle of animal models of DMD. Moreover, a few studies employing genetic mouse models have revealed that different MMPs play distinct roles in disease progression in DMD. Modulation of the activity of MMPs improves myofiber regeneration and enhances the efficacy of transplantation and engraftment of muscle progenitor cells in dystrophic muscle in mouse models of DMD. Furthermore, recent reports also suggest that some MMPs especially MMP-9 can serve as a biomarker for diagnosis and prognosis of DMD. In this article, we provide a succinct overview of the regulation of various MMPs and their therapeutic importance in DMD. Frontiers Media S.A. 2014-04-01 /pmc/articles/PMC4207008/ /pubmed/25364719 http://dx.doi.org/10.3389/fcell.2014.00011 Text en Copyright © 2014 Ogura, Tajrishi, Sato, Hindi and Kumar. http://creativecommons.org/licenses/by/3.0/ This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) or licensor are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms. |
spellingShingle | Cell and Developmental Biology Ogura, Yuji Tajrishi, Marjan M. Sato, Shuichi Hindi, Sajedah M. Kumar, Ashok Therapeutic potential of matrix metalloproteinases in Duchenne muscular dystrophy |
title | Therapeutic potential of matrix metalloproteinases in Duchenne muscular dystrophy |
title_full | Therapeutic potential of matrix metalloproteinases in Duchenne muscular dystrophy |
title_fullStr | Therapeutic potential of matrix metalloproteinases in Duchenne muscular dystrophy |
title_full_unstemmed | Therapeutic potential of matrix metalloproteinases in Duchenne muscular dystrophy |
title_short | Therapeutic potential of matrix metalloproteinases in Duchenne muscular dystrophy |
title_sort | therapeutic potential of matrix metalloproteinases in duchenne muscular dystrophy |
topic | Cell and Developmental Biology |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4207008/ https://www.ncbi.nlm.nih.gov/pubmed/25364719 http://dx.doi.org/10.3389/fcell.2014.00011 |
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