Brief Report

Loss of A-type Lamin Expression Compromises Nuclear Envelope Integrity Leading to Muscular Dystrophy

Correspondence to: Colin L. Stewart, Laboratory of Cancer and Developmental Biology, ABL-Basic Research Program, NCI-FCRDC, P.O. Box B, Frederick, MD 21702. Tel:(301) 846-1755 Fax:301-846-7117 E-mail:stewartc{at}mail.ncifcrf.gov.

The nuclear lamina is a protein meshwork lining the nucleoplasmic face of the inner nuclear membrane and represents an important determinant of interphase nuclear architecture. Its major components are the A- and B-type lamins. Whereas B-type lamins are found in all mammalian cells, A-type lamin expression is developmentally regulated. In the mouse, A-type lamins do not appear until midway through embryonic development, suggesting that these proteins may be involved in the regulation of terminal differentiation. Here we show that mice lacking A-type lamins develop to term with no overt abnormalities. However, their postnatal growth is severely retarded and is characterized by the appearance of muscular dystrophy. This phenotype is associated with ultrastructural perturbations to the nuclear envelope. These include the mislocalization of emerin, an inner nuclear membrane protein, defects in which are implicated in Emery-Dreifuss muscular dystrophy (EDMD), one of the three major X-linked dystrophies. Mice lacking the A-type lamins exhibit tissue-specific alterations to their nuclear envelope integrity and emerin distribution. In skeletal and cardiac muscles, this is manifest as a dystrophic condition related to EDMD.

Key Words: emerin, muscular dystrophy, nuclear envelope, lamins

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• Navarro, C. L., De Sandre-Giovannoli, A., Bernard, R., Boccaccio, I., Boyer, A., Genevieve, D., Hadj-Rabia, S., Gaudy-Marqueste, C., Smitt, H. S., Vabres, P., Faivre, L., Verloes, A., Van Essen, T., Flori, E., Hennekam, R., Beemer, F. A., Laurent, N., Le Merrer, M., Cau, P., Levy, N. (2004). Lamin A and ZMPSTE24 (FACE-1) defects cause nuclear disorganization and identify restrictive dermopathy as a lethal neonatal laminopathy. Hum Mol Genet 13: 2493-2503 [Abstract] [Full Text]  
• Tulac, S., Dosiou, C., Suchanek, E., Giudice, L. C. (2004). Silencing lamin A/C in human endometrial stromal cells: a model to investigate endometrial gene function and regulation. Mol Hum Reprod 10: 705-711 [Abstract] [Full Text]  
• Masny, P. S., Bengtsson, U., Chung, S.-A., Martin, J. H., van Engelen, B., van der Maarel, S. M., Winokur, S. T. (2004). Localization of 4q35.2 to the nuclear periphery: is FSHD a nuclear envelope disease?. Hum Mol Genet 13: 1857-1871 [Abstract] [Full Text]  
• Vergnes, L., Peterfy, M., Bergo, M. O., Young, S. G., Reue, K. (2004). Lamin B1 is required for mouse development and nuclear integrity. Proc. Natl. Acad. Sci. USA 101: 10428-10433 [Abstract] [Full Text]  
• Johnson, B. R., Nitta, R. T., Frock, R. L., Mounkes, L., Barbie, D. A., Stewart, C. L., Harlow, E., Kennedy, B. K. (2004). A-type lamins regulate retinoblastoma protein function by promoting subnuclear localization and preventing proteasomal degradation. Proc. Natl. Acad. Sci. USA 101: 9677-9682 [Abstract] [Full Text]  
• Gerace, L. (2004). TorsinA and torsion dystonia: Unraveling the architecture of the nuclear envelope. Proc. Natl. Acad. Sci. USA 101: 8839-8840 [Full Text]  
• Takano, M., Koyama, Y., Ito, H., Hoshino, S., Onogi, H., Hagiwara, M., Furukawa, K., Horigome, T. (2004). Regulation of Binding of Lamin B Receptor to Chromatin by SR Protein Kinase and cdc2 Kinase in Xenopus Egg Extracts. J. Biol. Chem. 279: 13265-13271 [Abstract] [Full Text]  
• Zastrow, M. S., Vlcek, S., Wilson, K. L. (2004). Proteins that bind A-type lamins: integrating isolated clues. J. Cell Sci. 117: 979-987 [Abstract] [Full Text]  
• Alsheimer, M., Liebe, B., Sewell, L., Stewart, C. L., Scherthan, H., Benavente, R. (2004). Disruption of spermatogenesis in mice lacking A-type lamins. J. Cell Sci. 117: 1173-1178 [Abstract] [Full Text]  
• Favreau, C., Higuet, D., Courvalin, J.-C., Buendia, B. (2004). Expression of a Mutant Lamin A That Causes Emery-Dreifuss Muscular Dystrophy Inhibits In Vitro Differentiation of C2C12 Myoblasts. Mol. Cell. Biol. 24: 1481-1492 [Abstract] [Full Text]  
• Maske, C. P., Hollinshead, M. S., Higbee, N. C., Bergo, M. O., Young, S. G., Vaux, D. J. (2003). A carboxyl-terminal interaction of lamin B1 is dependent on the CAAX endoprotease Rce1 and carboxymethylation. JCB 162: 1223-1232 [Abstract] [Full Text]  

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