Transcriptomic study of progerin-expressing medial aortas

Hutchinson-Gilford progeria syndrome (HGPS) is a rare genetic disease which is characterized by premature ageing. Affected children show accelerated cardiovascular disease, including atherosclerosis and vascular smooth muscle cell (VSMC) loss, and die at an average age of 14.6 years from myocardial infarction or stroke. The objective of this study was to detect the primary mechanism leading to VSMC death and accelerated atherosclerosis in mouse models of HGPS. In our RNA sequencing experiment we compared transcriptomes of medial aortas from both ubiquitous and VSMC-specific progeric models with its corresponding controls expressing lamin A/C or lamin C only, respectively. Our studies might not only help to find a cure for HGPS but also shed some light on physiological ageing.

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