Editorial Volume 10, Issue 10 pp 2555—2556

Accelerated atherosclerosis in HGPS

Figure 1. Proposed model of accelerated atherosclerosis induced by progerin expression in vascular smooth muscle cells (VSMCs). The aortic wall has three layers: the adventitia, media (containing VSMCs), and intima (containing endothelial cells). Progerin expression in VSMCs leads to their progressive loss in the aortic media and their replacement by extracellular matrix. These changes, together with other factors, increase low-density lipoprotein (LDL) retention in the aortic wall, which exacerbates atheroma plaque formation. In parallel, the adventitia becomes thicker and denser. Depletion of VSMCs in the atheroma may cause plaque instability and rupture, which ultimately provokes ischemic cardiovascular events (e.g. myocardial infarction) and premature death.