1ifr

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[[Image:1ifr.jpg|left|200px]]
[[Image:1ifr.jpg|left|200px]]
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{{Structure
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The line below this paragraph, containing "STRUCTURE_1ifr", creates the "Structure Box" on the page.
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{{STRUCTURE_1ifr| PDB=1ifr | SCENE= }}
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|RESOURCES=<span class='plainlinks'>[http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=1ifr FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=1ifr OCA], [http://www.ebi.ac.uk/pdbsum/1ifr PDBsum], [http://www.rcsb.org/pdb/explore.do?structureId=1ifr RCSB]</span>
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'''Structure of Lamin A/C Globular Domain'''
'''Structure of Lamin A/C Globular Domain'''
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[[Category: Shoelson, S E.]]
[[Category: Shoelson, S E.]]
[[Category: Werner, E D.]]
[[Category: Werner, E D.]]
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[[Category: immunoglobulin]]
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[[Category: Immunoglobulin]]
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''Page seeded by [http://oca.weizmann.ac.il/oca OCA ] on Fri May 2 19:57:02 2008''
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''Page seeded by [http://oca.weizmann.ac.il/oca OCA ] on Sun Mar 30 21:19:12 2008''
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Revision as of 16:57, 2 May 2008

Template:STRUCTURE 1ifr

Structure of Lamin A/C Globular Domain


Overview

The nuclear lamins form a two-dimensional matrix that provides integrity to the cell nucleus and participates in nuclear activities. Mutations in the region of human LMNA encoding the carboxyl-terminal tail Lamin A/C are associated with forms of muscular dystrophy and familial partial lipodystrophy (FPLD). To help discriminate tissue-specific phenotypes, we have solved at 1.4-A resolution the three-dimensional crystal structure of the lamin A/C globular tail. The domain adopts a novel, all beta immunoglobulin-like fold. FPLD-associated mutations cluster within a small surface, whereas muscular dystrophy-associated mutations are distributed throughout the protein core and on its surface. These findings distinguish myopathy- and lipodystrophy-associated mutations and provide a structural framework for further testing hypotheses concerning lamin function.

About this Structure

1IFR is a Single protein structure of sequence from Homo sapiens. Full crystallographic information is available from OCA.

Reference

Structure of the globular tail of nuclear lamin., Dhe-Paganon S, Werner ED, Chi YI, Shoelson SE, J Biol Chem. 2002 May 17;277(20):17381-4. Epub 2002 Mar 18. PMID:11901143 Page seeded by OCA on Fri May 2 19:57:02 2008

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