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IPSC derived cardiac fibroblasts of DMD patients show compromised actin microfilaments, metabolic shift and pro-fibrotic phenotype
Duchenne muscular dystrophy (DMD) is a severe form of muscular dystrophy caused by mutations in the dystrophin gene. We characterized which isoforms of dystrophin were expressed by human induced pluripotent stem cell (hiPSC)-derived cardiac fibroblasts obtained from control and DMD patients. Distinc...
Autores principales: | Soussi, Salwa, Savchenko, Lesia, Rovina, Davide, Iacovoni, Jason S., Gottinger, Andrea, Vialettes, Maxime, Pioner, Josè-Manuel, Farini, Andrea, Mallia, Sara, Rabino, Martina, Pompilio, Giulio, Parini, Angelo, Lairez, Olivier, Gowran, Aoife, Pizzinat, Nathalie |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
BioMed Central
2023
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10373315/ https://www.ncbi.nlm.nih.gov/pubmed/37501163 http://dx.doi.org/10.1186/s13062-023-00398-2 |
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