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.

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.