Progerin Expression in Endothelial Cells Induces Endothelial Dysfunction

ABSTRACT: Hutchinson-Gilford Progeria Syndrome (HGPS) is a rare, genetic premature aging disorder associated with severe atherosclerosis, often resulting in fatal heart attacks and strokes. Progerin, the mutant protein in HGPS, also is expressed in healthy individuals and may play a role in the development of atherosclerosis during physiologic aging. Here, we provide evidence for a primary involvement of vascular endothelium in the pathogenesis of accelerated atherosclerosis in HGPS. Expression of progerin in cultured human endothelial cells induces a dysfunctional phenotype, manifested by activation of multiple pro-inflammatory, pro-atherogenic genes. In particular, our data implicate endothelial-derived interleukin-1 (IL-1) as a key mediator of a pro-inflammatory vascular phenotype. Endothelia

ORGANISM(S): Homo sapiens

SUBMITTER: Belinda Yap 

PROVIDER: E-GEOD-19270 | biostudies-arrayexpress |

REPOSITORIES: biostudies-arrayexpress