Roles of the lamin A-specific tail region in the localization to sites of nuclear envelope rupture.

ABSTRACT: The nuclear lamina (NL) lines the nuclear envelope (NE) to maintain nuclear structure in metazoan cells. The major NL components, the nuclear lamins contribute to the protection against NE rupture induced by mechanical stress. Lamin A (LA) and a short form of the splicing variant lamin C (LC) are diffused from the nucleoplasm to sites of NE rupture in immortalized mouse embryonic fibroblasts (MEFs). LA localization to the rupture sites is significantly slow and weak compared with LC, but the underlying mechanism remains unknown. In this study, wild-type (WT), Hutchinson-Gilford Progeria syndrome (HGPS) knock-in MEFs expressing progerin (PG, an LA mutant lacking the second proteolytic cleavage site), and LA/C-knockout MEFs transiently and heterogeneously expressing LA/C WTs and mutants fuse

SUBMITTER: Kono Y 

PROVIDER: S-EPMC11645434 | biostudies-literature | 2024 Dec

REPOSITORIES: biostudies-literature