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00925njm 22002777a 4500 dc Trigueros-Motos, Laia author Gonzalez, Jose M author Rivera-Torres, Jose author Andres, Vicente author 2011-06-01 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. 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, cardiovascular disease and oxidative stress