Hutchinson-Gilford progeria syndrome (HGPS) is a fatal disease characterized by premature aging in which young children fail to thrive and adolescents die from myocardial infarction or stroke. The pathogenesis of HGPS is studied intensively because the mechanisms of premature aging may lead to a better understanding of normal aging. In this issue of the JCI, Osmanagic-Myers and colleagues identify the cellular mechanisms that lead to vascular abnormalities and death in children with HGPS.
Charles J. Lowenstein, J. Allen Bennett
In healthy endothelial cells, shear stress activates the mechanoresponsive myocardin-related transcription factor MRTF-A, increasing eNOS expression and NO production, which in turn inhibits tissue fibrosis.