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© The Rockefeller University Press, 0021-9525/1999//913 $5.00
The Journal of Cell Biology, Volume 147, Number 5, , 1999 913-920

Loss of a-Type Lamin Expression Compromises Nuclear Envelope Integrity Leading to Muscular Dystrophy

^a Advanced BioScience Laboratories–Basic Research Program, National Cancer Institute–Frederick Cancer Research and Development Center, Frederick, Maryland 21702
^b Hoffmann-LaRoche, Nutley, New Jersey 07110
^c Science Applications International Corporation, National Cancer Institute–Frederick Cancer Research and Development Center, Frederick, Maryland 21702
^d Department of Cell Biology and Anatomy, University of Calgary, Faculty of Medicine, Calgary, Canada T2N 4N1
Laboratory of Cancer and Developmental Biology, ABL-Basic Research Program, NCI-FCRDC, P.O. Box B, Frederick, MD 21702.301-846-7117(301) 846-1755

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

© 1999 The Rockefeller University Press

Abbreviations used in this paper: EC, embryonal carcinoma; EDMD, Emery-Dreifuss muscular dystrophy; ES, embryonic stem cell; INM, inner nuclear membrane; MEF, mouse embryonic fibroblasts; NE, nuclear envelope; NPC, nuclear pore complex.

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• Vantyghem, M. C., Pigny, P., Maurage, C. A., Rouaix-Emery, N., Stojkovic, T., Cuisset, J. M., Millaire, A., Lascols, O., Vermersch, P., Wemeau, J. L., Capeau, J., Vigouroux, C. (2004). Patients with Familial Partial Lipodystrophy of the Dunnigan Type Due to a LMNA R482W Mutation Show Muscular and Cardiac Abnormalities. J. Clin. Endocrinol. Metab. 89: 5337-5346 [Abstract] [Full Text]  
• Broers, J. L.V., Peeters, E. A.G., Kuijpers, H. J.H., Endert, J., Bouten, C. V.C., Oomens, C. W.J., Baaijens, F. P.T., Ramaekers, F. C.S. (2004). Decreased mechanical stiffness in LMNA-/- cells is caused by defective nucleo-cytoskeletal integrity: implications for the development of laminopathies. Hum Mol Genet 13: 2567-2580 [Abstract] [Full Text]  
• Vecerova, J., Koberna, K., Malinsky, J., Soutoglou, E., Sullivan, T., Stewart, C. L., Raska, I., Misteli, T. (2004). Formation of Nuclear Splicing Factor Compartments Is Independent of Lamins A/C. Mol. Biol. Cell 15: 4904-4910 [Abstract] [Full Text]  
• Schirmer, E. C., Gerace, L. (2004). The Stability of the Nuclear Lamina Polymer Changes with the Composition of Lamin Subtypes According to Their Individual Binding Strengths. J. Biol. Chem. 279: 42811-42817 [Abstract] [Full Text]  
• Muchir, A., Worman, H. J. (2004). The Nuclear Envelope and Human Disease. Physiology 19: 309-314 [Abstract] [Full Text]  
• 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]  

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