dc.contributor.author | Benedicto, I | en_US |
dc.contributor.author | Carmona, RM | en_US |
dc.contributor.author | Barettino, A | en_US |
dc.contributor.author | Espinós-Estévez, C | en_US |
dc.contributor.author | Gonzalo, P | en_US |
dc.contributor.author | Nevado, RM | en_US |
dc.contributor.author | de la Fuente-Pérez, M | en_US |
dc.contributor.author | Andrés-Manzano, MJ | en_US |
dc.contributor.author | González-Gómez, C | en_US |
dc.contributor.author | Rolas, L | en_US |
dc.contributor.author | Dorado, B | en_US |
dc.contributor.author | Nourshargh, S | en_US |
dc.contributor.author | Hamczyk, MR | en_US |
dc.contributor.author | Andrés, V | en_US |
dc.date.accessioned | 2024-04-03T09:47:21Z | |
dc.date.available | 2024-04-01 | en_US |
dc.identifier.issn | 0027-8424 | en_US |
dc.identifier.uri | https://qmro.qmul.ac.uk/xmlui/handle/123456789/95878 | |
dc.description.abstract | 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. To study atherosclerosis, we generated atheroprone mouse models by overexpressing a PCSK9 gain-of-function mutant. While HGPSrev-Cdh5-CreERT2 and HGPSrev mice developed a similar level of excessive atherosclerosis, plaque development in HGPSrev-SM22α-Cre mice was reduced to wild-type levels. Our studies demonstrate that progerin suppression in VSMCs, but not in ECs, prevents exacerbated atherosclerosis in progeroid mice. | en_US |
dc.language.iso | en | en_US |
dc.publisher | National Academy of Sciences | en_US |
dc.relation.ispartof | Proceedings of the National Academy of Sciences | en_US |
dc.rights | This open access article is distributed under Creative Commons Attribution-NonCommercial-NoDerivatives License 4.0 (CC BY-NC-ND). | |
dc.title | Exacerbated atherosclerosis in progeria is prevented by progerin elimination in vascular smooth muscle cells but not endothelial cells | en_US |
dc.type | Article | |
dc.rights.holder | © 2024 the Author(s). Published by PNAS. | |
pubs.notes | Not known | en_US |
pubs.publication-status | Accepted | en_US |
dcterms.dateAccepted | 2024-04-01 | en_US |
rioxxterms.funder | Default funder | en_US |
rioxxterms.identifier.project | Default project | en_US |