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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Bibliographic Details

Published in:	Biology Direct
Main Authors:	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
Format:	Article
Language:	English
Published:	BMC 2023-07-01
Subjects:	Duchenne Human induced pluripotent stem cell Fibroblasts Mitochondrial oxidation Actin microfilaments
Online Access:	https://doi.org/10.1186/s13062-023-00398-2