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...

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Detalles Bibliográficos
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
Formato: Online Artículo Texto
Lenguaje:English
Publicado: BioMed Central 2023
Materias:
Research
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

Internet

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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