Article
Nuclear membrane ruptures underlie the vascular pathology in a mouse model of Hutchinson-Gilford progeria syndrome.
JCI insight - 23 Aug 2021
Kim Paul H, Chen Natalie Y, Heizer Patrick J, Tu Yiping, Weston Thomas A, Fong Jared L-C, Gill Navjot Kaur, Rowat Amy C, Young Stephen G, Fong Loren G
Abstract excerpt
The mutant nuclear lamin protein (progerin) produced in Hutchinson-Gilford progeria syndrome (HGPS) results in loss of arterial smooth muscle cells (SMCs), but the mechanism has been unclear. We found that progerin induces repetitive nuclear membrane (NM) ruptures, DNA damage, and cell death in cultured SMCs. Reducing lamin B1 expression and exposing cells to mechanical stress - to mirror conditions in the aorta...
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