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NF2 : The wizardry of merlin
Author(s) -
Xiao GuangHui,
Chernoff Jonathan,
Testa Joseph R.
Publication year - 2003
Publication title -
genes, chromosomes and cancer
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.754
H-Index - 119
eISSN - 1098-2264
pISSN - 1045-2257
DOI - 10.1002/gcc.10282
Subject(s) - merlin (protein) , neurofibromatosis type 2 , radixin , moesin , ezrin , biology , carcinogenesis , microbiology and biotechnology , tumor suppressor gene , cancer research , neurofibromatosis , genetics , gene , cell , cytoskeleton , suppressor
Neurofibromatosis type II (NF2) is an autosomal dominant cancer syndrome characterized by the formation of tumors of the nervous system, particularly schwannomas and meningiomas. The NF2 gene is also implicated in the development of sporadic schwannomas and meningiomas, as well as tumor types seemingly unrelated to the NF2 disorder, such as malignant mesotheliomas. Inactivation of NF2 occurs by a “two‐hit” mechanism, as proposed by Al Knudson, and the NF2 gene behaves as a classical tumor suppressor gene. The NF2 gene product, merlin, exhibits homology with the ezrin–radixin–moesin family of membrane–cytoskeleton‐linking proteins. During the past several years, there has been intensive investigation aimed at elucidating the mechanisms underlying merlin's functions. In this review, we summarize the involvement of NF2 inactivation in tumorigenesis. We also discuss observations implicating merlin in cell motility and cell proliferation, with a focus on recent findings linking merlin to Rac signaling. © 2003 Wiley‐Liss, Inc.