Article
Progerin induces a phenotypic switch in vascular smooth muscle cells and triggers replication stress and an aging-associated secretory signature.
GeroScience - 1 Apr 2023
Coll-Bonfill Nuria, Mahajan Urvashi, Shashkova Elena V, Lin Chien-Jung, Mecham Robert P, Gonzalo Susana
Abstract excerpt
Hutchinson-Gilford progeria syndrome is a premature aging disease caused by LMNA gene mutation and the production of a truncated prelamin A protein "progerin" that elicits cellular and organismal toxicity. Progerin accumulates in the vasculature, being especially detrimental for vascular smooth muscle cells (VSMC). Vessel stiffening and aortic atherosclerosis in HGPS patients are accompanied by VSMC depletion in...
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