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''' | '''LEM DOMAIN OF HUMAN INNER NUCLEAR MEMBRANE PROTEIN EMERIN''' | ||
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==About this Structure== | ==About this Structure== | ||
- | 1JEI is a [[Single protein]] structure | + | 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.]] | ||
- | [[Category: | + | [[Category: Emerin nucleus membrane domain dystrophy]] |
- | + | ''Page seeded by [http://oca.weizmann.ac.il/oca OCA ] on Fri May 2 21:07:31 2008'' | |
- | ''Page seeded by [http://oca.weizmann.ac.il/oca OCA ] on | + |
Revision as of 18:07, 2 May 2008
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