1jei
From Proteopedia
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| - | [[Image:1jei.jpg|left|200px]] | + | [[Image:1jei.jpg|left|200px]] |
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| - | '''LEM DOMAIN OF HUMAN INNER NUCLEAR MEMBRANE PROTEIN EMERIN''' | + | {{Structure |
| + | |PDB= 1jei |SIZE=350|CAPTION= <scene name='initialview01'>1jei</scene> | ||
| + | |SITE= | ||
| + | |LIGAND= | ||
| + | |ACTIVITY= | ||
| + | |GENE= | ||
| + | }} | ||
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| + | '''LEM DOMAIN OF HUMAN INNER NUCLEAR MEMBRANE PROTEIN EMERIN''' | ||
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==Overview== | ==Overview== | ||
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==About this Structure== | ==About this Structure== | ||
| - | 1JEI is a [ | + | 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]. |
==Reference== | ==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:[http:// | + | 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:[http://www.ncbi.nlm.nih.gov/pubmed/11470279 11470279] |
[[Category: Single protein]] | [[Category: Single protein]] | ||
[[Category: Callebaut, I.]] | [[Category: Callebaut, I.]] | ||
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[[Category: emerin nucleus membrane domain dystrophy]] | [[Category: emerin nucleus membrane domain dystrophy]] | ||
| - | ''Page seeded by [http://oca.weizmann.ac.il/oca OCA ] on Thu | + | ''Page seeded by [http://oca.weizmann.ac.il/oca OCA ] on Thu Mar 20 12:02:19 2008'' |
Revision as of 10:02, 20 March 2008
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LEM DOMAIN OF HUMAN INNER NUCLEAR MEMBRANE PROTEIN EMERIN
Contents |
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.
Disease
Known diseases associated with this structure: Emery-Dreifuss muscular dystrophy OMIM:[300384]
About this Structure
1JEI is a Single protein structure of sequence from [1]. 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
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