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- Title
Abnormal nuclear shape and impaired mechanotransduction in emerin-deficient cells.
- Authors
Lammerding, Jan; Hsiao, Janet; Schulze, P. Christian; Kozlov, Serguei; Stewart, Cohn L.; Lee, Richard T.
- Abstract
Emery-Dreifuss muscular dystrophy can be caused by mutations in the nuclear envelope proteins lamin A/C and emerin. We recently demonstrated that A-type lamin-deficient cells have impaired nuclear mechanics and altered mechanotransduction, suggesting two potential disease mechanisms (Lammerding, J., P.C. Schulze, T. Takahashi, S. Kozlov, T. Sullivan, R.D. Kamm, C.L. Stewart, and R.T. Lee. 2004. J. Clin. Invest 113: 370-378). Here, we examined the function of emerin on nuclear mechanics and strain-induced signaling. Emerin-deficient mouse embryo fibroblasts have abnormal nuclear shape, but in contrast to A-type lamin-deficient cells, exhibit nuclear deformations comparable to wild-type cells in cellular strain experiments, and the integrity of emerin-deficient nuclear envelopes appeared normal in a nuclear microinjection assay. Interestingly, expression of mechanosensitive genes in response to mechanical strain was impaired in emerin-deficient cells, and prolonged mechanical stimulation increased apoptosts in emerin-deficient cells. Thus, emerin-deficient mouse embryo fibroblasts have apparently normal nuclear mechanics but impaired expression of mechanosensitive genes in response to strain, suggesting that emerin mutations may act through altered transcriptional regulation and not by increasing nuclear fragility.
- Subjects
MUSCULAR dystrophy; NEUROMUSCULAR diseases; FIBROBLASTS; GENETIC transduction; MICROBIAL genetics; GENES
- Publication
Journal of Cell Biology, 2005, Vol 170, Issue 5, p781
- ISSN
0021-9525
- Publication type
Article
- DOI
10.1083/jcb.200502148