Vascular cell types in progeria: victims or villains?

Ignacio Benedicto, Magda R Hamczyk, Beatriz Dorado, Vicente Andrés

Centro de Investigaciones Biológicas Margarita Salas (CIB), Consejo Superior de Investigaciones Científicas (CSIC), 28040 Madrid, Spain; Centro Nacional de Investigaciones Cardiovasculares (CNIC), 28029 Madrid, Spain. Electronic address: [email protected] de Investigaciones Biológicas Margarita Salas (CIB), Consejo Superior de Investigaciones Científicas (CSIC), 28040 Madrid, Spain; Centro Nacional de Investigaciones Cardiovasculares (CNIC), 28029 Madrid, Spain. Electronic address: [email protected]. · pubmed

Paper

Abstract

Hutchinson-Gilford progeria syndrome (HGPS) is an ultrarare genetic disease caused by progerin, a broadly expressed mutant variant of lamin A protein that accelerates aging and leads to premature death typically in adolescence. Progerin affects many organs and reproduces many characteristics of physiological aging, with the main cause of death in HGPS being atherosclerotic cardiovascular disease (CVD). Due to the rarity of HGPS, advances in understanding the disease and progress toward new therapeutic approaches are crucially dependent on preclinical models. We discuss recent research developments from a variety of HGPS experimental systems, with a special focus on in vivo studies of the role of vascular smooth muscle cells (VSMCs) and endothelial cells (ECs) that are key players in atherosclerosis.

The following analysis is generated by AI and may contain errors. It is separate from the authors’ abstract and is not peer review.

Research summary

The paper discusses the role of vascular smooth muscle cells and endothelial cells in Hutchinson-Gilford progeria syndrome, highlighting their involvement in atherosclerosis and aging-related cardiovascular disease. This research is relevant as it addresses mechanisms underlying aging and potential therapeutic targets that could influence longevity.

Author of this paper? Send a correction.