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J. Biol. Chem., Vol. 277, Issue 12, 10332-10336, March 22, 2002
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From the Neurofibromatosis type 2 (NF2) is a dominantly
inherited disease associated with the central nervous system. The NF2
gene product merlin is a tumor suppressor, and its mutation or
inactivation causes this disease. We report here the crystal structure
of the merlin FERM domain containing a 22-residue The atomic coordinates and the structure factors (code 1ISN) have been deposited in the Protein Data Bank, Research Collaboratory for Structural Bioinformatics, Rutgers University, New Brunswick, NJ (http://www.rcsb.org/).
Structural Basis for Neurofibromatosis Type 2
CRYSTAL STRUCTURE OF THE MERLIN FERM DOMAIN*
,,,§,
, and**
Structural Biology Laboratory, Nara
Institute of Science and Technology and ** CREST, Japan
Science and Technology Corporation, 8916-5 Takayama, Ikoma, Nara
630-0101, Japan, the ¶ Department of Cell Biology, Faculty of
Medicine, Kyoto University, Sakyo-ku, Kyoto 606-8501, Japan, and the
College of Medical Technology, Kyoto University, Sakyo-ku, Kyoto
606-8507, Japan
-helical segment. The structure reveals that the merlin FERM domain consists of three
subdomains displaying notable features of the electrostatic surface
potentials, although the overall surface potentials similar to those of
ezrin/radixin/moesin (ERM) proteins indicate electrostatic membrane
association. The structure also is consistent with inactivation mechanisms caused by the pathogenic mutations associated with NF2.
*
This work was supported by Grant-in-aid for Scientific
Research 12490024 (to T. H.) from JSPS and Grant-in-aid for
Scientific Research on Priority Area 10179104 (to S. T. and T. H.)
from the MECSST of Japan.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.
Member of the Structural Biology Sakabe Project of the
Foundation for Advancement of International Science. To whom
correspondence should be addressed. Tel.: 81-0743-72-5570; Fax:
81-0743-72-5579; E-mail: hakosima@bs.aist-nara.ac.jp.
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