The fibrotic niche impairs satellite cell function and muscle regeneration in mouse models of Marfan syndrome.

Meiricris Tomaz da SilvaAudrei R SantosTatiana E KoikeTabata L NascimentoAndrei RozanskiDarko BosnakovskiLygia V PereiraAshok KumarMichael KybaElen H Miyabara

Published in: Acta physiologica (Oxford, England) (2022)

The fibrotic niche, caused by Fbn1 mutations, reduces the myogenic potential of satellite cells, affecting structural and functional muscle regeneration. In addition, the fibrosis inhibitor Ang-(1-7) partially counteracts satellite cell abnormalities and restores myofiber size and contractile force in regenerating muscles.

Keyphrases

skeletal muscle
stem cells
induced apoptosis
systemic sclerosis
mouse model
idiopathic pulmonary fibrosis
single cell
cell cycle arrest
cell therapy
angiotensin ii
single molecule
wound healing
case report
oxidative stress
risk assessment
signaling pathway
mesenchymal stem cells