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.
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29 August 2005
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August 22 2005
Abnormal nuclear shape and impaired mechanotransduction in emerin-deficient cells
Jan Lammerding,
Jan Lammerding
1Cardiovascular Division, Brigham and Women's Hospital, Boston, MA 02115
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Janet Hsiao,
Janet Hsiao
2HST Division, Massachusetts Institute of Technology, Cambridge, MA 02139
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P. Christian Schulze,
P. Christian Schulze
1Cardiovascular Division, Brigham and Women's Hospital, Boston, MA 02115
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Serguei Kozlov,
Serguei Kozlov
3Cancer and Developmental Biology Lab, National Cancer Institute, Frederick, MD 21702
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Colin L. Stewart,
Colin L. Stewart
3Cancer and Developmental Biology Lab, National Cancer Institute, Frederick, MD 21702
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Richard T. Lee
Richard T. Lee
1Cardiovascular Division, Brigham and Women's Hospital, Boston, MA 02115
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Jan Lammerding
1Cardiovascular Division, Brigham and Women's Hospital, Boston, MA 02115
Janet Hsiao
2HST Division, Massachusetts Institute of Technology, Cambridge, MA 02139
P. Christian Schulze
1Cardiovascular Division, Brigham and Women's Hospital, Boston, MA 02115
Serguei Kozlov
3Cancer and Developmental Biology Lab, National Cancer Institute, Frederick, MD 21702
Colin L. Stewart
3Cancer and Developmental Biology Lab, National Cancer Institute, Frederick, MD 21702
Richard T. Lee
1Cardiovascular Division, Brigham and Women's Hospital, Boston, MA 02115
Correspondence to Jan Lammerding: [email protected]
Abbreviations used in this paper: BAF, barrier-to-autointegration factor; EDMD, Emery-Dreifuss muscular dystrophy; GAPDH, glyceraldehyde 3-phosphate dehydrogenase; GCL, germ cell-less.
Received:
February 24 2005
Accepted:
July 25 2005
Online ISSN: 1540-8140
Print ISSN: 0021-9525
Government
2005
J Cell Biol (2005) 170 (5): 781–791.
Article history
Received:
February 24 2005
Accepted:
July 25 2005
Citation
Jan Lammerding, Janet Hsiao, P. Christian Schulze, Serguei Kozlov, Colin L. Stewart, Richard T. Lee; Abnormal nuclear shape and impaired mechanotransduction in emerin-deficient cells . J Cell Biol 29 August 2005; 170 (5): 781–791. doi: https://doi.org/10.1083/jcb.200502148
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