2026-08-05T15:42:45Zhttps://repisalud.isciii.es/rest/oai/requestoai:repisalud.isciii.es:20.500.12105/74042024-09-27T08:40:05Zcom_20.500.12105_19604com_20.500.12105_2051col_20.500.12105_19605
Repisalud
author
Hamczyk, Magda R.
author
Andres, Vicente
funder
Ministerio de Ciencia, Innovación y Universidades (España)
funder
Unión Europea. Fondo Europeo de Desarrollo Regional (FEDER/ERDF)
funder
Progeria Research Foundation
funder
Fundación La Marató TV3
funder
Fundación ProCNIC
funder
Instituto de Salud Carlos III
2019-03-27T09:12:23Z
2019-03-27T09:12:23Z
2019
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
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.
eng
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
journal article
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URL
https://repisalud.isciii.es/bitstreams/5a9add4a-77dc-483f-8294-59a5d4586a33/download
File
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VascularSmoothMuscleCell_2019.pdf
URL
https://repisalud.isciii.es/bitstreams/cbd42302-0b81-402a-8f7a-fee8a6b8d167/download
File
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VascularSmoothMuscleCell_2019.pdf.txt