Article
IPSC derived cardiac fibroblasts of DMD patients show compromised actin microfilaments, metabolic shift and pro-fibrotic phenotype.
Biology direct - 27 Jul 2023
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
Abstract excerpt
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. Distinct dystrophin isoforms were observed; however, highest molecular weight isoform was absent in DMD patients carrying...
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