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

Abstract 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...

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Principais autores:	Luca Caputo, Alice Granados, Jessica Lenzi, Alessandro Rosa, Slimane Ait-Si-Ali, Pier Lorenzo Puri, Sonia Albini
Formato:	Artigo
Idioma:	Inglês
Publicado:	BMC 2020-05-01
Series:	Skeletal Muscle
Assuntos:	Duchenne muscular dystrophy iPSC TGFβ pSMAD
Acceso en liña:	http://link.springer.com/article/10.1186/s13395-020-00224-7
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