Article
ESCRT-III controls nuclear envelope deformation induced by progerin.
Scientific reports - 2 Nov 2020
Arii Jun, Maeda Fumio, Maruzuru Yuhei, Koyanagi Naoto, Kato Akihisa, Mori Yasuko, Kawaguchi Yasushi
Abstract excerpt
Hutchinson-Gilford progeria syndrome (HGPS) is a premature aging disorder, caused by mutation in the gene encoding lamin A/C, which produces a truncated protein called progerin. In cells from HGPS patients, progerin accumulates at the nuclear membrane (NM), where it causes NM deformations. In this study, we investigated whether progerin-induced NM deformation involved ESCRT-III, a protein complex that remodels...
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