Vascular Smooth Muscle-Specific Progerin Expression Accelerates Atherosclerosis and Death in a Mouse Model of Hutchinson-Gilford Progeria Syndrome.

ABSTRACT:

Background

Progerin, an aberrant protein that accumulates with age, causes the rare genetic disease Hutchinson-Gilford progeria syndrome (HGPS). Patients who have HGPS exhibit ubiquitous progerin expression, accelerated aging and atherosclerosis, and die in their early teens, mainly of myocardial infarction or stroke. The mechanisms underlying progerin-induced atherosclerosis remain unexplored, in part, because of the lack of appropriate animal models.

Methods

We generated an atherosclerosis-prone model of HGPS by crossing apolipoprotein E-deficient (Apoe^-/-) mice with Lmna^G609G/G609G mice ubiquitously expressing progerin. To induce progerin expression specifically in macrophages or vascular smooth muscle cells (VSMCs), we crossed <

SUBMITTER: Hamczyk MR 

PROVIDER: S-EPMC6075893 | biostudies-literature | 2018 Jul

REPOSITORIES: biostudies-literature