Endoplasmic Reticulum Stress at the Crossroads of Progeria and Atherosclerosis
March 2019
in “
EMBO molecular medicine
”
TLDR A defective protein in progeria causes cell death and atherosclerosis, but a treatment targeting cell stress may reduce these effects.
The document reviews a study by Hamczyk et al. (2019) on Hutchinson-Gilford progeria syndrome (HGPS), a condition marked by rapid aging and early death due to cardiovascular issues like atherosclerosis. It explains that progerin, a defective protein, leads to the death of vascular smooth muscle cells and endothelial cells, contributing to atherosclerosis. The study identified that endoplasmic reticulum (ER) stress and the unfolded protein response (UPR) are significantly disrupted in HGPS, playing a key role in cell death and the progression of atherosclerosis. Furthermore, the study demonstrated that treatment with tauroursodeoxycholic acid (TUDCA), a chemical chaperone, in mice with VSMC-specific progerin expression, lessened atherosclerosis and extended lifespan, indicating that targeting ER stress and UPR could be an effective treatment for atherosclerosis in HGPS and possibly in normal aging.
