Human Molecular Genetics, Vol 5, 801-808, Copyright © 1996 by Oxford University Press
S Manilal, TM Nguyen, CA Sewry and GE Morris
A large fragment of emerin cDNA was prepared by PCR and expressed as a
recombinant protein in Escherichia coli. Using this as immunogen, we
prepared a panel of 12 monoclonal antibodies which recognise at least four
different epitopes on emerin in order to ensure that emerin can be
distinguished from non-specific cross-reacting proteins. All the mAbs
recognised a 34 kDa protein in all tissues tested, though minor emerin-
related bands were also detected in some tissues. Immunofluorescence
microscopy showed that emerin is located at the nuclear rim in all tissues
examined. A muscle biopsy from an Emery-Dreifuss muscular dystrophy (EMDM)
patient showed complete absence of emerin by both Western blotting and
immunohistochemistry, suggesting a simple diagnostic antibody test for EDMD
families. Biochemical fractionation of brain and liver tissues showed that
emerin was present in nuclei purified by centrifugation through 65% sucrose
and was absent from soluble fractions (post-100,000 g). From these results,
together with sequence and structural homologies between emerin,
thymopoietins and the nuclear lamina-associated protein, LAP2, we suggest
that emerin will prove to be one member of a family of inner nuclear
membrane proteins.
ARTICLES
The Emery-Dreifuss muscular dystrophy protein, emerin, is a nuclear membrane protein
MRIC Biotechnology Group, N.E. Wales Institute, UK.
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S. Martins, T Eide, R. Steen, T Jahnsen, S Skalhegg B, and P Collas HA95 is a protein of the chromatin and nuclear matrix regulating nuclear envelope dynamics J. Cell Sci., January 11, 2000; 113(21): 3703 - 3713. [Abstract] [PDF]
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J Gotzmann, S Vlcek, and R Foisner Caspase-mediated cleavage of the chromosome-binding domain of lamina-associated polypeptide 2 alpha J. Cell Sci., January 11, 2000; 113(21): 3769 - 3780. [Abstract] [PDF]
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T Haraguchi, T Koujin, T Hayakawa, T Kaneda, C Tsutsumi, N Imamoto, C Akazawa, J Sukegawa, Y Yoneda, and Y Hiraoka Live fluorescence imaging reveals early recruitment of emerin, LBR, RanBP2, and Nup153 to reforming functional nuclear envelopes J. Cell Sci., January 3, 2000; 113(5): 779 - 794. [Abstract] [PDF]
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G. E. Morris and S. Manilal Heart to heart: from nuclearproteins to Emery-Dreifuss muscular dystrophy Hum. Mol. Genet., September 1, 1999; 8(10): 1847 - 1851. [Abstract] [Full Text] [PDF]
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M. M. Rolls, P. A. Stein, S. S. Taylor, E. Ha, F. McKeon, and T. A. Rapoport A Visual Screen of a Gfp-Fusion Library Identifies a New Type of Nuclear Envelope Membrane Protein J. Cell Biol., July 12, 1999; 146(1): 29 - 44. [Abstract] [Full Text] [PDF]
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T. M. Gant, C. A. Harris, and K. L. Wilson Roles of LAP2 Proteins in Nuclear Assembly and DNA Replication: Truncated LAP2{beta} Proteins Alter Lamina Assembly, Envelope Formation, Nuclear Size, and DNA Replication Efficiency in Xenopus laevis Extracts J. Cell Biol., March 22, 1999; 144(6): 1083 - 1096. [Abstract] [Full Text] [PDF]
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E. Fairley, J Kendrick-Jones, and J. Ellis The Emery-Dreifuss muscular dystrophy phenotype arises from aberrant targeting and binding of emerin at the inner nuclear membrane J. Cell Sci., January 8, 1999; 112(15): 2571 - 2582. [Abstract] [PDF]
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C Ostlund, J Ellenberg, E Hallberg, J Lippincott-Schwartz, and H. Worman Intracellular trafficking of emerin, the Emery-Dreifuss muscular dystrophy protein J. Cell Sci., January 6, 1999; 112(11): 1709 - 1719. [Abstract] [PDF]
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K. Furukawa, C. E. Fritze, and L. Gerace The Major Nuclear Envelope Targeting Domain of LAP2 Coincides with Its Lamin Binding Region but Is Distinct from Its Chromatin Interaction Domain J. Biol. Chem., February 13, 1998; 273(7): 4213 - 4219. [Abstract] [Full Text] [PDF]
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J. Ellis, M Craxton, J. Yates, and J Kendrick-Jones Aberrant intracellular targeting and cell cycle-dependent phosphorylation of emerin contribute to the Emery-Dreifuss muscular dystrophy phenotype J. Cell Sci., January 3, 1998; 111(6): 781 - 792. [Abstract] [PDF]
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