Ignacio Benedicto, Rosa M Carmona, Ana Barettino, Carla Espinós-Estévez, Pilar Gonzalo, Rosa M Nevado, Miguel de la Fuente-Pérez, María J Andrés-Manzano, Cristina González-Gómez, Loïc Rolas, Beatriz Dorado, Sussan Nourshargh, Magda R Hamczyk, Vicente Andrés
Hutchinson-Gilford progeria syndrome (HGPS) is a rare disease caused by the expression of progerin, a mutant protein that accelerates aging and precipitates death. Given that atherosclerosis complications are the main cause of death in progeria, here, we investigated whether progerin-induced atherosclerosis is prevented in HGPSrev-Cdh5-CreERT2 and HGPSrev-SM22α-Cre mice with progerin suppression in endothelial cells (ECs) and vascular smooth muscle cells (VSMCs), respectively. HGPSrev-Cdh5-CreERT2 mice were undistinguishable from HGPSrev mice with ubiquitous progerin expression, in contrast with the ameliorated progeroid phenotype of HGPSrev-SM22α-Cre mice...
April 30, 2024: Proceedings of the National Academy of Sciences of the United States of America