1ivt
From Proteopedia
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[[Image:1ivt.gif|left|200px]] | [[Image:1ivt.gif|left|200px]] | ||
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'''NMR structures of the C-terminal globular domain of human lamin A/C''' | '''NMR structures of the C-terminal globular domain of human lamin A/C''' | ||
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[[Category: Worman, H J.]] | [[Category: Worman, H J.]] | ||
[[Category: Zinn-Justin, S.]] | [[Category: Zinn-Justin, S.]] | ||
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| - | [[Category: | + | [[Category: Ig-fold]] |
| - | + | ''Page seeded by [http://oca.weizmann.ac.il/oca OCA ] on Fri May 2 20:28:48 2008'' | |
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Revision as of 17:28, 2 May 2008
NMR structures of the C-terminal globular domain of human lamin A/C
Overview
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.
About this Structure
1IVT is a Single protein structure of sequence from Homo sapiens. Full crystallographic 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 Fri May 2 20:28:48 2008
