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1jei

From Proteopedia

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[[Image:1jei.jpg|left|200px]]
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{{Seed}}
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[[Image:1jei.png|left|200px]]
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{{STRUCTURE_1jei| PDB=1jei | SCENE= }}
{{STRUCTURE_1jei| PDB=1jei | SCENE= }}
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'''LEM DOMAIN OF HUMAN INNER NUCLEAR MEMBRANE PROTEIN EMERIN'''
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===LEM DOMAIN OF HUMAN INNER NUCLEAR MEMBRANE PROTEIN EMERIN===
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==Overview==
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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.
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The line below this paragraph, {{ABSTRACT_PUBMED_11470279}}, adds the Publication Abstract to the page
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(as it appears on PubMed at http://www.pubmed.gov), where 11470279 is the PubMed ID number.
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{{ABSTRACT_PUBMED_11470279}}
==About this Structure==
==About this Structure==
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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].
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1JEI is a [[Single protein]] structure. Full experimental information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=1JEI OCA].
==Reference==
==Reference==
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[[Category: Zinn-Justin, S.]]
[[Category: Zinn-Justin, S.]]
[[Category: Emerin nucleus membrane domain dystrophy]]
[[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 Tue Jul 1 20:07:19 2008''

Revision as of 17:07, 1 July 2008

Image:1jei.png

Template:STRUCTURE 1jei

LEM DOMAIN OF HUMAN INNER NUCLEAR MEMBRANE PROTEIN EMERIN

Template:ABSTRACT PUBMED 11470279

About this Structure

1JEI is a Single protein structure. Full experimental 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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