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A Spotlight on T Lymphocytes in Duchenne Muscular Dystrophy—Not Just a Muscle Defect

The lack of dystrophin in Duchenne muscular dystrophy (DMD) results in membrane fragility resulting in contraction-induced muscle damage and subsequent inflammation. The impact of inflammation is profound, resulting in fibrosis of skeletal muscle, the diaphragm and heart, which contributes to muscle...

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Detalles Bibliográficos
Autores principales: Coles, Chantal A., Woodcock, Ian, Pellicci, Daniel G., Houweling, Peter J.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8945129/
https://www.ncbi.nlm.nih.gov/pubmed/35327337
http://dx.doi.org/10.3390/biomedicines10030535
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author Coles, Chantal A.
Woodcock, Ian
Pellicci, Daniel G.
Houweling, Peter J.
author_facet Coles, Chantal A.
Woodcock, Ian
Pellicci, Daniel G.
Houweling, Peter J.
author_sort Coles, Chantal A.
collection PubMed
description The lack of dystrophin in Duchenne muscular dystrophy (DMD) results in membrane fragility resulting in contraction-induced muscle damage and subsequent inflammation. The impact of inflammation is profound, resulting in fibrosis of skeletal muscle, the diaphragm and heart, which contributes to muscle weakness, reduced quality of life and premature death. To date, the innate immune system has been the major focus in individuals with DMD, and our understanding of the adaptive immune system, specifically T cells, is limited. Targeting the immune system has been the focus of multiple clinical trials for DMD and is considered a vital step in the development of better treatments. However, we must first have a complete picture of the involvement of the immune systems in dystrophic muscle disease to better understand how inflammation influences disease progression and severity. This review focuses on the role of T cells in DMD, highlighting the importance of looking beyond skeletal muscle when considering how the loss of dystrophin impacts disease progression. Finally, we propose that targeting T cells is a potential novel therapeutic in the treatment of DMD.
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spelling pubmed-89451292022-03-25 A Spotlight on T Lymphocytes in Duchenne Muscular Dystrophy—Not Just a Muscle Defect Coles, Chantal A. Woodcock, Ian Pellicci, Daniel G. Houweling, Peter J. Biomedicines Review The lack of dystrophin in Duchenne muscular dystrophy (DMD) results in membrane fragility resulting in contraction-induced muscle damage and subsequent inflammation. The impact of inflammation is profound, resulting in fibrosis of skeletal muscle, the diaphragm and heart, which contributes to muscle weakness, reduced quality of life and premature death. To date, the innate immune system has been the major focus in individuals with DMD, and our understanding of the adaptive immune system, specifically T cells, is limited. Targeting the immune system has been the focus of multiple clinical trials for DMD and is considered a vital step in the development of better treatments. However, we must first have a complete picture of the involvement of the immune systems in dystrophic muscle disease to better understand how inflammation influences disease progression and severity. This review focuses on the role of T cells in DMD, highlighting the importance of looking beyond skeletal muscle when considering how the loss of dystrophin impacts disease progression. Finally, we propose that targeting T cells is a potential novel therapeutic in the treatment of DMD. MDPI 2022-02-24 /pmc/articles/PMC8945129/ /pubmed/35327337 http://dx.doi.org/10.3390/biomedicines10030535 Text en © 2022 by the authors. https://creativecommons.org/licenses/by/4.0/Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (https://creativecommons.org/licenses/by/4.0/).
spellingShingle Review
Coles, Chantal A.
Woodcock, Ian
Pellicci, Daniel G.
Houweling, Peter J.
A Spotlight on T Lymphocytes in Duchenne Muscular Dystrophy—Not Just a Muscle Defect
title A Spotlight on T Lymphocytes in Duchenne Muscular Dystrophy—Not Just a Muscle Defect
title_full A Spotlight on T Lymphocytes in Duchenne Muscular Dystrophy—Not Just a Muscle Defect
title_fullStr A Spotlight on T Lymphocytes in Duchenne Muscular Dystrophy—Not Just a Muscle Defect
title_full_unstemmed A Spotlight on T Lymphocytes in Duchenne Muscular Dystrophy—Not Just a Muscle Defect
title_short A Spotlight on T Lymphocytes in Duchenne Muscular Dystrophy—Not Just a Muscle Defect
title_sort spotlight on t lymphocytes in duchenne muscular dystrophy—not just a muscle defect
topic Review
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8945129/
https://www.ncbi.nlm.nih.gov/pubmed/35327337
http://dx.doi.org/10.3390/biomedicines10030535
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