Myopathologic trajectory in Duchenne muscular dystrophy (DMD) reveals lack of regeneration due to senescence in satellite cells Article Swipe
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· 2023
· Open Access
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· DOI: https://doi.org/10.1186/s40478-023-01657-z
Duchenne muscular dystrophy (DMD) is a devastating X-linked muscular disease, caused by mutations in the DMD gene encoding Dystrophin and affecting 1:5000 boys worldwide. Lack of Dystrophin leads to progressive muscle wasting and degeneration resulting in cardiorespiratory failure. Despite the absence of a definitive cure, innovative therapeutic avenues are emerging. Myopathologic studies are important to further understand the biological mechanisms of the disease and to identify histopathologic benchmarks for clinical evaluations. We conducted a myopathologic analysis on twenty-four muscle biopsies from DMD patients, with particular emphasis on regeneration, fibro-adipogenic progenitors and muscle stem cells behavior. We describe an increase in content of fibro-adipogenic progenitors, central orchestrators of fibrotic progression and lipid deposition, concurrently with a decline in muscle regenerative capacity. This regenerative impairment strongly correlates with compromised activation and expansion of muscle stem cells. Furthermore, our study uncovers an early acquisition of a senescence phenotype by DMD-afflicted muscle stem cells. Here we describe the myopathologic trajectory intrinsic to DMD and establish muscle stem cell senescence as a pivotal readout for future therapeutic interventions.
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- article
- Language
- en
- Landing Page
- https://doi.org/10.1186/s40478-023-01657-z
- https://actaneurocomms.biomedcentral.com/counter/pdf/10.1186/s40478-023-01657-z
- OA Status
- gold
- Cited By
- 33
- References
- 50
- Related Works
- 10
- OpenAlex ID
- https://openalex.org/W4387776447
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- OpenAlex ID
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https://openalex.org/W4387776447Canonical identifier for this work in OpenAlex
- DOI
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https://doi.org/10.1186/s40478-023-01657-zDigital Object Identifier
- Title
-
Myopathologic trajectory in Duchenne muscular dystrophy (DMD) reveals lack of regeneration due to senescence in satellite cellsWork title
- Type
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articleOpenAlex work type
- Language
-
enPrimary language
- Publication year
-
2023Year of publication
- Publication date
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2023-10-19Full publication date if available
- Authors
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Nastasia Cardone, Valentina Taglietti, Serena Baratto, Kaouthar Kefi, Baptiste Periou, Cyril Gitiaux, Christine Barnérias, Peggy Lafuste, France Leturcq Pharm, Juliette Nectoux Pharm, Chiara Panicucci, Isabelle Desguerre, Claudio Bruno, François‐Jérôme Authier, Chiara Fiorillo, Frédéric Relaix, Edoardo MalfattiList of authors in order
- Landing page
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https://doi.org/10.1186/s40478-023-01657-zPublisher landing page
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https://actaneurocomms.biomedcentral.com/counter/pdf/10.1186/s40478-023-01657-zDirect link to full text PDF
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YesWhether a free full text is available
- OA status
-
goldOpen access status per OpenAlex
- OA URL
-
https://actaneurocomms.biomedcentral.com/counter/pdf/10.1186/s40478-023-01657-zDirect OA link when available
- Concepts
-
Duchenne muscular dystrophy, Wasting, Stem cell, Dystrophin, Medicine, Senescence, mdx mouse, Regeneration (biology), Muscular dystrophy, Regenerative medicine, Progenitor cell, Myocyte, Bioinformatics, Neuroscience, Pathology, Biology, Cell biology, Internal medicineTop concepts (fields/topics) attached by OpenAlex
- Cited by
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33Total citation count in OpenAlex
- Citations by year (recent)
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2025: 18, 2024: 13, 2023: 2Per-year citation counts (last 5 years)
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50Number of works referenced by this work
- Related works (count)
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10Other works algorithmically related by OpenAlex
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| sustainable_development_goals[0].display_name | Good health and well-being |
| citation_normalized_percentile.value | 0.96982289 |
| citation_normalized_percentile.is_in_top_1_percent | False |
| citation_normalized_percentile.is_in_top_10_percent | True |