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IPSC derived cardiac fibroblasts of DMD patients show compromised actin microfilaments, metabolic shift and pro-fibrotic phenotype

Abstract 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...

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Principais autores: Salwa Soussi, Lesia Savchenko, Davide Rovina, Jason S. Iacovoni, Andrea Gottinger, Maxime Vialettes, Josè-Manuel Pioner, Andrea Farini, Sara Mallia, Martina Rabino, Giulio Pompilio, Angelo Parini, Olivier Lairez, Aoife Gowran, Nathalie Pizzinat
Formato: Artigo
Idioma:Inglês
Publicado em: BMC 2023-07-01
coleção:Biology Direct
Assuntos:
Duchenne
Human induced pluripotent stem cell
Fibroblasts
Mitochondrial oxidation
Actin microfilaments
Acesso em linha:https://doi.org/10.1186/s13062-023-00398-2
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