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Papers In Press, published online ahead of print December 27, 2001
Yokohama City University, Yokohama 230-0045
Corresponding Author: shimizu{at}tsurumi.yokohama-cu.ac.jp
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 a-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 its electrostatic membrane association. The structure also suggests the inactivation mechanisms caused by the pathogenic mutations associated with NF2.
J. Biol. Chem, 10.1074/jbc.M109979200
Submitted on October 16, 2001
Revised on December 14, 2001
Accepted on December 27, 2001
Structural basis for neurofibromatosis type 2: Crystal structure of the merlin FERM domain
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