1jei

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[[Image:1jei.jpg|left|200px]]
[[Image:1jei.jpg|left|200px]]
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{{Structure
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|PDB= 1jei |SIZE=350|CAPTION= <scene name='initialview01'>1jei</scene>
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The line below this paragraph, containing "STRUCTURE_1jei", creates the "Structure Box" on the page.
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{{STRUCTURE_1jei| PDB=1jei | SCENE= }}
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|RELATEDENTRY=[[1h9e|1H9E]], [[1h9f|1H9F]]
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|RESOURCES=<span class='plainlinks'>[http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=1jei FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=1jei OCA], [http://www.ebi.ac.uk/pdbsum/1jei PDBsum], [http://www.rcsb.org/pdb/explore.do?structureId=1jei RCSB]</span>
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'''LEM DOMAIN OF HUMAN INNER NUCLEAR MEMBRANE PROTEIN EMERIN'''
'''LEM DOMAIN OF HUMAN INNER NUCLEAR MEMBRANE PROTEIN EMERIN'''
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==About this Structure==
==About this Structure==
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1JEI is a [[Single protein]] structure of sequence from [http://en.wikipedia.org/wiki/ ]. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=1JEI OCA].
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1JEI is a [[Single protein]] structure. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=1JEI OCA].
==Reference==
==Reference==
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[[Category: Wolff, N.]]
[[Category: Wolff, N.]]
[[Category: Zinn-Justin, S.]]
[[Category: Zinn-Justin, S.]]
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[[Category: emerin nucleus membrane domain dystrophy]]
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[[Category: Emerin nucleus membrane domain dystrophy]]
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''Page seeded by [http://oca.weizmann.ac.il/oca OCA ] on Fri May 2 21:07:31 2008''
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''Page seeded by [http://oca.weizmann.ac.il/oca OCA ] on Sun Mar 30 21:32:32 2008''
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Revision as of 18:07, 2 May 2008

Template:STRUCTURE 1jei

LEM DOMAIN OF HUMAN INNER NUCLEAR MEMBRANE PROTEIN EMERIN


Overview

Like Duchenne and Becker muscular dystrophies, Emery-Dreifuss muscular dystrophy (EDMD) is characterized by myopathic and cardiomyopathic abnormalities. EDMD has the particularity of being linked to mutations in nuclear proteins. The X-linked form of EDMD is caused by mutations in the emerin gene, whereas autosomal dominant EDMD is caused by mutations in the lamin A/C gene. Emerin colocalizes with lamin A/C in interphase cells, and binds in vitro to lamin A/C. Recent work suggests that lamin A/C might serve as a receptor for emerin. We have undertaken a structural analysis of emerin, and in particular of its N-terminal domain, which is comprised in the emerin segment critical for binding to lamin A/C. We show that region 2-54 of emerin adopts the LEM fold. This fold was originally described in the two N-terminal domains of another inner nuclear membrane protein called lamina-associated protein 2 (LAP2). The existence of a conserved solvent-exposed surface on the LEM domains of LAP2 and emerin is discussed, as well as the nature of a possible common target.

About this Structure

1JEI is a Single protein structure. Full crystallographic information is available from OCA.

Reference

Structural analysis of emerin, an inner nuclear membrane protein mutated in X-linked Emery-Dreifuss muscular dystrophy., Wolff N, Gilquin B, Courchay K, Callebaut I, Worman HJ, Zinn-Justin S, FEBS Lett. 2001 Jul 20;501(2-3):171-6. PMID:11470279 Page seeded by OCA on Fri May 2 21:07:31 2008

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