Progerin in muscle leads to thermogenic and metabolic defects via impaired calcium homeostasis

Volume: 19, Issue: 2
Published: Dec 12, 2019
Abstract
Mutations in lamin A ( LMNA ) are responsible for a variety of human dystrophic and metabolic diseases. Here, we created a mouse model in which progerin, the lamin A mutant protein that causes Hutchinson–Gilford progeria syndrome (HGPS), can be inducibly overexpressed. Muscle‐specific overexpression of progerin was sufficient to induce muscular dystrophy and alter whole‐body energy expenditure, leading to premature death. Intriguingly,...
Paper Details
Title
Progerin in muscle leads to thermogenic and metabolic defects via impaired calcium homeostasis
Published Date
Dec 12, 2019
Journal
Volume
19
Issue
2
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