Cargando…

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...

Descripción completa

Detalles Bibliográficos
Autores principales: Kodippili, Kasun, Rudnicki, Michael A.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Frontiers Media S.A. 2023
Materias:
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
_version_ 1785058728957968384
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
work_keys_str_mv AT kodippilikasun satellitecellcontributiontodiseasepathologyinduchennemusculardystrophy
AT rudnickimichaela satellitecellcontributiontodiseasepathologyinduchennemusculardystrophy