Por favor, use este identificador para citar o enlazar este Item:http://hdl.handle.net/20.500.12105/15201
Título
Molecular and Cellular Mechanisms Driving Cardiovascular Disease in Hutchinson-Gilford Progeria Syndrome: Lessons Learned from Animal Models.
Autor(es)
Fecha de publicación
2021
Cita
Cells. 2021 May 11;10(5):1157.
Idioma
Inglés
Tipo de documento
journal article
Resumen
Hutchinson-Gilford progeria syndrome (HGPS) is a rare genetic disease that recapitulates many symptoms of physiological aging and precipitates death. Patients develop severe vascular alterations, mainly massive vascular smooth muscle cell loss, vessel stiffening, calcification, fibrosis, and generalized atherosclerosis, as well as electrical, structural, and functional anomalies in the heart. As a result, most HGPS patients die of myocardial infarction, heart failure, or stroke typically during the first or second decade of life. No cure exists for HGPS, and therefore it is of the utmost importance to define the mechanisms that control disease progression in order to develop new treatments to improve the life quality of patients and extend their lifespan. Since the discovery of the HGPS-causing mutation, several animal models have been generated to study multiple aspects of the syndrome and to analyze the contribution of different cell types to the acquisition of the HGPS-associated cardiovascular phenotype. This review discusses current knowledge about cardiovascular features in HGPS patients and animal models and the molecular and cellular mechanisms through which progerin causes cardiovascular disease.
MESH
Disease Models, Animal | Aging | Animals | Atherosclerosis | Cardiovascular Diseases | Cardiovascular System | Clinical Trials as Topic | Cytoskeleton | Endothelium, Vascular | Fibrosis | Heart Diseases | Humans | Lamin Type A | Mice | Muscle, Smooth | Muscle, Smooth, Vascular | Myocardial Infarction | Phenotype | Progeria | Stroke | Vascular Calcification
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- Nombre:
- Cells 2021 May 11 10 Molecular ...
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- 980.3Kb
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