New vascular insights into premature aging
Charles J. Lowenstein, J. Allen Bennett
Charles J. Lowenstein, J. Allen Bennett
Published December 18, 2018
Citation Information: J Clin Invest. 2018. https://doi.org/10.1172/JCI125616.
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Category: Commentary

New vascular insights into premature aging

Abstract

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.

Authors

Charles J. Lowenstein, J. Allen Bennett

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Figure 1

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.

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In healthy endothelial cells, shear stress activates the mechanoresponsi...
However, in endothelial cells of subjects with Hutchinson-Gilford progeria syndrome, the mutant lamin called progerin interferes with normal mechanosensing, inhibits eNOS expression and NO synthesis, and permits accelerated fibrosis.