2026-06-14T05:06:25Zhttps://repisalud.isciii.es/rest/oai/requestoai:repisalud.isciii.es:20.500.12105/76592024-09-27T08:25:37Zcom_20.500.12105_19604com_20.500.12105_2051col_20.500.12105_19605
Repisalud
author
Trigueros-Motos, Laia
author
Gonzalez, Jose M
author
Rivera-Torres, Jose
author
Andres, Vicente
funder
Ministerio de Ciencia e Innovación (España)
funder
Unión Europea. Fondo Europeo de Desarrollo Regional (FEDER/ERDF)
funder
Instituto de Salud Carlos III
funder
Fundación Ramón Areces
funder
Fundación ProCNIC
2019-05-23T09:00:09Z
2019-05-23T09:00:09Z
2011-06-01
Front Biosci (Schol Ed). 2011; 3(3):1285-97
10.2741/226
1945-0524
Frontiers in bioscience (Scholar edition)
21622271
http://hdl.handle.net/20.500.12105/7659
Hutchinson-Gilford Progeria Syndrome (HGPS), a rare human disease characterized by premature aging, is mainly caused by the abnormal accumulation of progerin, a mutant form of the mammalian nuclear envelope component lamin A. HGPS patients exhibit vascular alterations and die at an average age of 13 years, predominantly from myocardial infarction or stroke. Animal models of HGPS have been a valuable tool in the study of the pathological processes implicated in the origin of this disease and its associated cardiovascular alterations. Some of the molecular mechanisms of HGPS might be relevant to the process of normal aging, since progerin is detected in cells from normal elderly humans. Conversely, processes linked to normal aging, such as the increase in oxidative stress, might be relevant to the pathogenic mechanisms of HGPS. In this review, we discuss recent advances in the understanding of the molecular mechanisms underlying the cardiovascular alterations associated with HGPS, the potential role of oxidative stress, and therapeutic approaches for the treatment of this devastating disease.
eng
Hutchinson-Gilford progeria syndrome, cardiovascular disease and oxidative stress
journal article
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Hutchinson-GilfordProgeriaSyndrome_2011.pdf
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Hutchinson-GilfordProgeriaSyndrome_2011.pdf.txt