2026-10-18T20:56:27Zhttps://repisalud.isciii.es/rest/oai/requestoai:repisalud.isciii.es:20.500.12105/277542026-10-14T00:15:59Zcom_20.500.12105_19586com_20.500.12105_2202col_20.500.12105_19587
Repisalud
author
Benedicto, Ignacio
author
Hamczyk, Magda R
author
Dorado, Beatriz
author
Andrés, Vicente
2026-10-13T08:28:59Z
2026-10-13T08:28:59Z
2026-01
Trends Mol Med. 2026 Jan;32(1):34-48.
https://hdl.handle.net/20.500.12105/27754
40240194
10.1016/j.molmed.2025.03.005
1471-499X
Trends in Molecular Medicine
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.
eng
Hutchinson-Gilford progeria syndrome
atherosclerosis
cardiovascular disease
endothelial cells
progerin
vascular smooth muscle cells
Vascular cell types in progeria: victims or villains?
research article
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Vascular cell types in progeria_Trends Mol Med_2026.pdf