Por favor, use este identificador para citar o enlazar este Item:http://hdl.handle.net/20.500.12105/7659
Título
Hutchinson-Gilford progeria syndrome, cardiovascular disease and oxidative stress
Autor(es)
Trigueros-Motos, Laia CNIC | Gonzalez, Jose M CNIC | Rivera-Torres, Jose CNIC | Andres, Vicente CNIC
Fecha de publicación
2011-06-01
Cita
Front Biosci (Schol Ed). 2011; 3(3):1285-97
Idioma
Inglés
Tipo de documento
journal article
Resumen
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.
MESH
Aging | Alternative Splicing | Animals | Cardiovascular Diseases | Cholesterol | Diphosphonates | Genetic Therapy | Humans | Lamin Type A | Membrane Proteins | Metalloendopeptidases | Mice | Mice, Knockout | Nuclear Proteins | Oligonucleotides | Oxidative Stress | Progeria | Protein Precursors | Terpenes
Versión en línea
DOI
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