Progeria and Heart Disease: Understanding Their Link
Does Progeria Affect the Heart?
The most serious consequence of Hutchinson-Gilford Progeria Syndrome (HGPS) is the early onset of cardiovascular disease, leading to premature death at an average age of 14.5 years.
Causes of Hutchinson-Gilford Progeria Syndrome (HGPS)
HGPS is attributed to a mutation in the LMNA gene, resulting in the production of a toxic protein known as progerin. This disrupts normal cell function and accelerates aging, causing severe atherosclerosis by early teens.
How Endothelial Cells are Affected in HGPS?
The research team utilized advanced single-cell RNA sequencing to analyze gene expression changes in endothelial cells from both HGPS and healthy mouse models. Findings revealed a significant activation of the YAP/TAZ pathway within endothelial cells, promoting inflammation and contributing to the acceleration of atherosclerosis.
Promising Therapeutic Implications for Progeria
To test therapy possibilities, researchers inhibited the YAP/TAZ pathway using verteporfin, a drug already approved for other conditions. This led to observable reductions in atherosclerosis progression in murine models, inspiring hopes for future treatments not only for HGPS but also for related age-associated cardiovascular diseases.
This article was prepared using information from open sources in accordance with the principles of Ethical Policy. The editorial team is not responsible for absolute accuracy, as it relies on data from the sources referenced.