Por favor, use este identificador para citar o enlazar este Item:http://hdl.handle.net/20.500.12105/7404
Título
Vascular smooth muscle cell loss underpins the accelerated atherosclerosis in Hutchinson-Gilford progeria syndrome
Autor(es)
Hamczyk, Magda R. CNIC | Andres, Vicente CNIC
Fecha de publicación
2019
Cita
Nucleus. 2019; 10(1):28-34
Idioma
Inglés
Tipo de documento
journal article
Resumen
Lamin A, a product of the LMNA gene, is an essential nuclear envelope component in most differentiated cells. Mutations in LMNA have been linked to premature aging disorders, including Hutchinson-Gilford progeria syndrome (HGPS). HGPS is caused by progerin, an aberrant form of lamin A that leads to premature death, typically from the complications of atherosclerotic disease. A key characteristic of HGPS is a severe loss of vascular smooth muscle cells (VSMCs) in the arteries. Various mouse models of HGPS have been created, but few of them feature VSMC depletion and none develops atherosclerosis, the death-causing symptom of the disease in humans. We recently generated a mouse model that recapitulates most features of HGPS, including VSMC loss and accelerated atherosclerosis. Furthermore, by generating cell-type-specific HGPS mouse models, we have demonstrated a central role of VSMC loss in progerin-induced atherosclerosis and premature death.
Palabras clave
Atherosclerosis | Hutchinson-Gilford progeria syndrome | lamin A | progerin | vascular smooth muscle cells
Versión en línea
DOI
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- VascularSmoothMuscleCell_2019.pdf
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- Artículo