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...
| Main Authors: | , , , |
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| Format: | Article |
| Language: | English |
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MDPI AG
2022-02-01
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| Series: | Biomedicines |
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| Online Access: | https://www.mdpi.com/2227-9059/10/3/535 |
| _version_ | 1797472785400856576 |
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| author | Chantal A. Coles Ian Woodcock Daniel G. Pellicci Peter J. Houweling |
| author_facet | Chantal A. Coles Ian Woodcock Daniel G. Pellicci Peter J. Houweling |
| author_sort | Chantal A. Coles |
| collection | DOAJ |
| 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. |
| first_indexed | 2024-03-09T20:06:11Z |
| format | Article |
| id | doaj.art-c007b3482d9e4d0c834b85cb6762f072 |
| institution | Directory Open Access Journal |
| issn | 2227-9059 |
| language | English |
| last_indexed | 2024-03-09T20:06:11Z |
| publishDate | 2022-02-01 |
| publisher | MDPI AG |
| record_format | Article |
| series | Biomedicines |
| spelling | doaj.art-c007b3482d9e4d0c834b85cb6762f0722023-11-24T00:31:39ZengMDPI AGBiomedicines2227-90592022-02-0110353510.3390/biomedicines10030535A Spotlight on T Lymphocytes in Duchenne Muscular Dystrophy—Not Just a Muscle DefectChantal A. Coles0Ian Woodcock1Daniel G. Pellicci2Peter J. Houweling3Murdoch Children’s Research Institute (MCRI), Melbourne, VIC 3052, AustraliaMurdoch Children’s Research Institute (MCRI), Melbourne, VIC 3052, AustraliaMurdoch Children’s Research Institute (MCRI), Melbourne, VIC 3052, AustraliaMurdoch Children’s Research Institute (MCRI), Melbourne, VIC 3052, AustraliaThe 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.https://www.mdpi.com/2227-9059/10/3/535duchenne muscular dystrophyT cellsinflammationdystrophic thymusTregs |
| spellingShingle | Chantal A. Coles Ian Woodcock Daniel G. Pellicci Peter J. Houweling A Spotlight on T Lymphocytes in Duchenne Muscular Dystrophy—Not Just a Muscle Defect Biomedicines duchenne muscular dystrophy T cells inflammation dystrophic thymus Tregs |
| 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 | duchenne muscular dystrophy T cells inflammation dystrophic thymus Tregs |
| url | https://www.mdpi.com/2227-9059/10/3/535 |
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