Sci Adv·· 7 hours agoPickScore61
Muscle stem cells produce a protective Fibrillin-1 matrix to prevent precocious activation.
In brief
Fibrillin-1 (FBN1), a Notch-regulated matrix glycoprotein made by muscle stem cells themselves, is required to keep those cells quiescent. Deleting Fbn1 in muscle stem cells causes dose-dependent quiescence defects, loss of cellular projections, depletion of the stem cell pool, and progressive muscle decline, while human Marfan syndrome biopsies show abnormally activated satellite cells. TGFβ blockade rescues the defects of FBN1-deficient muscle, linking excess TGFβ signaling to stem cell dysfunction in MFS myopathy.
Why it matters
Identifies a cell-autonomous extracellular matrix mechanism of muscle stem cell quiescence and a druggable TGFβ pathway relevant to Marfan myopathy.
Source: Sci Adv · doi.org