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. 2019 Mar 22;11(4):e10360. doi: 10.15252/emmm.201910360

Figure 1. Mechanisms of VSMC loss in HGPS‐dependent atherosclerosis.

Figure 1

(A) State of the art of cellular and molecular mechanisms taking place in HGPS‐VSMCs from animal models and patients (either primary or iPSC‐derived cells). Nuclear structural defects (1), accumulation of progerin (2), transcriptional changes due to chromatin remodeling (3), and increased DNA damage (4) are the “classical” hallmarks of progeric cells. In addition, activation of the NRF2 pathway and oxidative stress are associated with HGPS. (B) New molecular insights from the work published in this issue, demonstrating that the ER stress response and activation of the UPR play major roles in VSMC death and atherosclerosis in mice with VSMC‐restricted progerin expression. Administration of the chemical chaperone tauroursodeoxycholic acid (TUDCA) is sufficient to rescue cell homeostasis, reduce atherosclerosis progression, and improve lifespan.