caa a22 4500 397575572 CHVBK 20180308164855.0 cr unu---uuuuu 161202e199606 xx s 000 0 eng 10.1093/hmg/5.6.801 doi (NATIONALLICENCE)oxford-10.1093/hmg/5.6.801 The Emery-Dreifuss Muscular Dystrophy Protein, Emerin, is a Nuclear Membrane Protein [Elektronische Daten] [S. Manilal, Nguyen thi Man, C. A. Sewry, G. E. 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 emerinrelated 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 immunohisto-chemistry, 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. © 1996 Oxford University Press Manilal S. MRIC Biotechnology Group, N.E. Wales Institute, Deeside, Clwyd CH5 4BR, UK aut Man Nguyen thi MRIC Biotechnology Group, N.E. Wales Institute, Deeside, Clwyd CH5 4BR, UK aut Sewry C. A. Neuromuscular Unit, Department of Paediatrics and Neonatal Medicine and Muscle Cell Biology Group, MRC Clinical Sciences Centre, Royal Postgraduate Medical School, Du Cane Road, London W12 ONN, UK aut Morris G. E. MRIC Biotechnology Group, N.E. Wales Institute, Deeside, Clwyd CH5 4BR, UK aut Human Molecular Genetics Oxford University Press 5/6(1996-06), 801-808 0964-6906 5:6<801 1996 5 hmg https://doi.org/10.1093/hmg/5.6.801 text/html Onlinezugriff via DOI 1 research-article jats NATIONALLICENCE 856 40 https://doi.org/10.1093/hmg/5.6.801 text/html Onlinezugriff via DOI NATIONALLICENCE 700 1- Manilal S. MRIC Biotechnology Group, N.E. Wales Institute, Deeside, Clwyd CH5 4BR, UK aut NATIONALLICENCE 700 1- Man Nguyen thi MRIC Biotechnology Group, N.E. Wales Institute, Deeside, Clwyd CH5 4BR, UK aut NATIONALLICENCE 700 1- Sewry C. A. Neuromuscular Unit, Department of Paediatrics and Neonatal Medicine and Muscle Cell Biology Group, MRC Clinical Sciences Centre, Royal Postgraduate Medical School, Du Cane Road, London W12 ONN, UK aut NATIONALLICENCE 700 1- Morris G. E. MRIC Biotechnology Group, N.E. Wales Institute, Deeside, Clwyd CH5 4BR, UK aut NATIONALLICENCE 773 0- Human Molecular Genetics Oxford University Press 5/6(1996-06), 801-808 0964-6906 5:6<801 1996 5 hmg Metadata rights reserved CC BY-NC-4.0 http://creativecommons.org/licenses/by-nc/4.0 nationallicence BK010053 XK010053 XK010000 NATIONALLICENCE NATIONALLICENCE NL-oxford