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1ivt

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[[Image:1ivt.gif|left|200px]]
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{{STRUCTURE_1ivt| PDB=1ivt | SCENE= }}
{{STRUCTURE_1ivt| PDB=1ivt | SCENE= }}
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'''NMR structures of the C-terminal globular domain of human lamin A/C'''
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===NMR structures of the C-terminal globular domain of human lamin A/C===
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==Overview==
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Lamins are nuclear intermediate filaments that, together with lamin-associated proteins, maintain nuclear shape and provide a structural support for chromosomes and replicating DNA. We have determined the solution structure of the human lamin A/C C-terminal globular domain which contains specific mutations causing four different heritable diseases. This domain encompasses residues 430-545 and adopts an Ig-like fold of type s. We have also characterized by NMR and circular dichroism the structure and thermostability of three mutants, R453W and R482W/Q, corresponding to "hot spots" causing Emery-Dreifuss muscular dystrophy and Dunnigan-type lipodystrophy, respectively. Our structure determination and mutant analyses clearly show that the consequences of the mutations causing muscle-specific diseases or lipodystrophy are different at the molecular level.
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(as it appears on PubMed at http://www.pubmed.gov), where 12057196 is the PubMed ID number.
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{{ABSTRACT_PUBMED_12057196}}
==About this Structure==
==About this Structure==
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1IVT is a [[Single protein]] structure of sequence from [http://en.wikipedia.org/wiki/Homo_sapiens Homo sapiens]. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=1IVT OCA].
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1IVT is a [[Single protein]] structure of sequence from [http://en.wikipedia.org/wiki/Homo_sapiens Homo sapiens]. Full experimental information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=1IVT OCA].
==Reference==
==Reference==
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[[Category: Beta barrel]]
[[Category: Beta barrel]]
[[Category: Ig-fold]]
[[Category: Ig-fold]]
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''Page seeded by [http://oca.weizmann.ac.il/oca OCA ] on Fri May 2 20:28:48 2008''
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''Page seeded by [http://oca.weizmann.ac.il/oca OCA ] on Tue Jul 1 14:05:24 2008''

Revision as of 11:05, 1 July 2008

Image:1ivt.png

Template:STRUCTURE 1ivt

NMR structures of the C-terminal globular domain of human lamin A/C

Template:ABSTRACT PUBMED 12057196

About this Structure

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

Reference

The Ig-like structure of the C-terminal domain of lamin A/C, mutated in muscular dystrophies, cardiomyopathy, and partial lipodystrophy., Krimm I, Ostlund C, Gilquin B, Couprie J, Hossenlopp P, Mornon JP, Bonne G, Courvalin JC, Worman HJ, Zinn-Justin S, Structure. 2002 Jun;10(6):811-23. PMID:12057196

Page seeded by OCA on Tue Jul 1 14:05:24 2008

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