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Originally published In Press as doi:10.1074/jbc.C200038200 on March 18, 2002

J. Biol. Chem., Vol. 277, Issue 20, 17381-17384, May 17, 2002
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ACCELERATED PUBLICATION
Structure of the Globular Tail of Nuclear Lamin*

Sirano Dhe-PaganonDagger , Eric D. Werner§, Young-In ChiDagger , and Steven E. Shoelson

From the Joslin Diabetes Center & Department of Medicine, Harvard Medical School, Boston, Massachusetts 02215

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-Å 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.

* This work was supported in part by National Institutes of Health Grant R01 DK43123.The costs of publication of this article were defrayed in part by the payment of page charges. The article must therefore be hereby marked "advertisement" in accordance with 18 U.S.C. Section 1734 solely to indicate this fact.

The atomic coordinates and the structure factors (code 1IFR) have been deposited in the Protein Data Bank, Research Collaboratory for Structural Bioinformatics, Rutgers University, New Brunswick, NJ (http://www.rcsb.org/).

Dagger Both authors supported by Mary K. Iacocca Fellowships.

§ Supported by National Institutes of Health Fellowship DK09393.

To whom correspondence should be addressed: Joslin Diabetes Center, One Joslin Place, Boston, MA 02215. E-mail: Steven. Shoelson@joslin.harvard.edu.

Copyright © 2002 by The American Society for Biochemistry and Molecular Biology, Inc.


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