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oai:repisalud.isciii.es:20.500.12105/74042024-09-27T08:40:05Zcom_20.500.12105_19604com_20.500.12105_2051col_20.500.12105_19605

00925njm 22002777a 4500 dc Hamczyk, Magda R. author Andres, Vicente author 2019 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. Nucleus. 2019; 10(1):28-34 10.1080/19491034.2019.1589359 1949-1042 1949-1034 Nucleus (Austin, Tex.) 30900948 http://hdl.handle.net/20.500.12105/7404 Atherosclerosis Hutchinson-Gilford progeria syndrome lamin A progerin vascular smooth muscle cells Vascular smooth muscle cell loss underpins the accelerated atherosclerosis in Hutchinson-Gilford progeria syndrome