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Published online 22 August 2005. doi:10.1083/jcb.200502148
The Rockefeller University Press, 0021-9525 $8.00
JCB, Volume 170, Number 5, 781-791
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Article

 Abnormal nuclear shape and impaired mechanotransduction in emerin-deficient cells

Jan Lammerding1, Janet Hsiao2, P. Christian Schulze1, Serguei Kozlov3, Colin L. Stewart3, and Richard T. Lee1

1 Cardiovascular Division, Brigham and Women's Hospital, Boston, MA 02115
2 HST Division, Massachusetts Institute of Technology, Cambridge, MA 02139
3 Cancer and Developmental Biology Lab, National Cancer Institute, Frederick, MD 21702

Correspondence to Jan Lammerding: jlammerding{at}rics.bwh.harvard.edu

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 apoptosis 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.

Abbreviations used in this paper: BAF, barrier-to-autointegration factor; EDMD, Emery-Dreifuss muscular dystrophy; GAPDH, glyceraldehyde 3-phosphate dehydrogenase; GCL, germ cell-less.


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