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

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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Bibliographic Details
Main Authors: Luca Caputo, Alice Granados, Jessica Lenzi, Alessandro Rosa, Slimane Ait-Si-Ali, Pier Lorenzo Puri, Sonia Albini
Format: Article
Language:English
Published: BMC 2020-05-01
Series:Skeletal Muscle
Subjects:
Duchenne muscular dystrophy
iPSC
TGFβ
pSMAD
Online Access:http://link.springer.com/article/10.1186/s13395-020-00224-7

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http://link.springer.com/article/10.1186/s13395-020-00224-7

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