The fibrotic niche impairs satellite cell function in regenerating muscles in mouse models of Marfan syndrome

This study investigates skeletal muscle regeneration in FBN1-deficient (MFS) mice. The results of a transplantation assay showed that regenerating muscles from MFS mice showed reduced satellite cell self-renewal, increased fibrosis, and impaired contractile function, and these effects were counteracted by angiotensin -1-7 [Ang-(1-7)]. These findings shed new light on the cellular and molecular mechanisms responsible for stem cell dysfunction in Marfan syndrome and provide a basis for developing novel effective strategies for preventing or treating muscle fibrosis.