Utilization of a NF2-Mutant Mouse Strain to Investigate the Cellular and Molecular Function of the NF2 Tumor Suppressor, Merlin
Annual rept. 15 Sep 2000-14 Sep 2001
MASSACHUSETTS GENERAL HOSPITAL BOSTON
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Neurofibromatosis type 2 NF2 is a familial cancer syndrome that features the development of nervous system tumors. The NF2-encoded protein, merlin, localizes to the membranecytoskeleton interface, raising the intriguing question of how a protein that occupies such a physical niche controls cell proliferation. To generate an animal model for NF2 and to build the foundation for delineating the molecular function of merlin, we established a Nf2-mutant mouse strain through genetic engineerins. Nf2- mice develop a spectrum of tumors that is distinct from that of their human counterparts, including osteosarcomas and hepatocellular carcinomas, which exhibit loss of the wild-type Nf2 allele.
- Anatomy and Physiology
- Medicine and Medical Research