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Satellite cell contribution to disease pathology in Duchenne muscular dystrophy
Progressive muscle weakness and degeneration characterize Duchenne muscular dystrophy (DMD), a lethal, x-linked neuromuscular disorder that affects 1 in 5,000 boys. Loss of dystrophin protein leads to recurrent muscle degeneration, progressive fibrosis, chronic inflammation, and dysfunction of skele...
Autores principales: | , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
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Frontiers Media S.A.
2023
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Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10266354/ https://www.ncbi.nlm.nih.gov/pubmed/37324396 http://dx.doi.org/10.3389/fphys.2023.1180980 |
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author | Kodippili, Kasun Rudnicki, Michael A. |
author_facet | Kodippili, Kasun Rudnicki, Michael A. |
author_sort | Kodippili, Kasun |
collection | PubMed |
description | Progressive muscle weakness and degeneration characterize Duchenne muscular dystrophy (DMD), a lethal, x-linked neuromuscular disorder that affects 1 in 5,000 boys. Loss of dystrophin protein leads to recurrent muscle degeneration, progressive fibrosis, chronic inflammation, and dysfunction of skeletal muscle resident stem cells, called satellite cells. Unfortunately, there is currently no cure for DMD. In this mini review, we discuss how satellite cells in dystrophic muscle are functionally impaired, and how this contributes to the DMD pathology, and the tremendous potential of restoring endogenous satellite cell function as a viable treatment strategy to treat this debilitating and fatal disease. |
format | Online Article Text |
id | pubmed-10266354 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2023 |
publisher | Frontiers Media S.A. |
record_format | MEDLINE/PubMed |
spelling | pubmed-102663542023-06-15 Satellite cell contribution to disease pathology in Duchenne muscular dystrophy Kodippili, Kasun Rudnicki, Michael A. Front Physiol Physiology Progressive muscle weakness and degeneration characterize Duchenne muscular dystrophy (DMD), a lethal, x-linked neuromuscular disorder that affects 1 in 5,000 boys. Loss of dystrophin protein leads to recurrent muscle degeneration, progressive fibrosis, chronic inflammation, and dysfunction of skeletal muscle resident stem cells, called satellite cells. Unfortunately, there is currently no cure for DMD. In this mini review, we discuss how satellite cells in dystrophic muscle are functionally impaired, and how this contributes to the DMD pathology, and the tremendous potential of restoring endogenous satellite cell function as a viable treatment strategy to treat this debilitating and fatal disease. Frontiers Media S.A. 2023-05-30 /pmc/articles/PMC10266354/ /pubmed/37324396 http://dx.doi.org/10.3389/fphys.2023.1180980 Text en Copyright © 2023 Kodippili and Rudnicki. https://creativecommons.org/licenses/by/4.0/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) and the copyright owner(s) 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 | Physiology Kodippili, Kasun Rudnicki, Michael A. Satellite cell contribution to disease pathology in Duchenne muscular dystrophy |
title | Satellite cell contribution to disease pathology in Duchenne muscular dystrophy |
title_full | Satellite cell contribution to disease pathology in Duchenne muscular dystrophy |
title_fullStr | Satellite cell contribution to disease pathology in Duchenne muscular dystrophy |
title_full_unstemmed | Satellite cell contribution to disease pathology in Duchenne muscular dystrophy |
title_short | Satellite cell contribution to disease pathology in Duchenne muscular dystrophy |
title_sort | satellite cell contribution to disease pathology in duchenne muscular dystrophy |
topic | Physiology |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10266354/ https://www.ncbi.nlm.nih.gov/pubmed/37324396 http://dx.doi.org/10.3389/fphys.2023.1180980 |
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