Acute conversion of patient-derived Duchenne muscular dystrophy iPSC into myotubes reveals constitutive and inducible over-activation of TGFβ-dependent pro-fibrotic signaling

BACKGROUND: In Duchenne muscular dystrophy (DMD), DYSTROPHIN deficiency exposes myofibers to repeated cycles of contraction/degeneration, ultimately leading to muscle loss and replacement by fibrotic tissue. DMD pathology is typically exacerbated by excessive secretion of TGFβ and consequent accumul...

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Detalles Bibliográficos
Autores principales: Caputo, Luca, Granados, Alice, Lenzi, Jessica, Rosa, Alessandro, Ait-Si-Ali, Slimane, Puri, Pier Lorenzo, Albini, Sonia
Formato: Online Artículo Texto
Lenguaje:English
Publicado: BioMed Central 2020
Materias:
Research
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7195779/
https://www.ncbi.nlm.nih.gov/pubmed/32359374
http://dx.doi.org/10.1186/s13395-020-00224-7

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