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J Biol Chem. 2002 Mar 22;277(12):10332-6. Epub 2001 Dec 27.

Structural basis for neurofibromatosis type 2. Crystal structure of the merlin FERM domain.

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  • 1Structural Biology Laboratory, Nara Institute of Science and Technology and CREST, Japan.


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 alpha-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.

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