NF2/merlin in hereditary neurofibromatosis 2 versus cancer: biologic mechanisms and clinical associations

Inactivating germline mutations in the tumor suppressor gene NF2 cause the hereditary syndrome neurofibromatosis 2, which is characterized by the development of neoplasms of the nervous system, most notably bilateral vestibular schwannoma. Somatic NF2 mutations have also been reported in a variety o...

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Published inOncotarget Vol. 5; no. 1; pp. 67 - 77
Main Authors Schroeder, Rebecca Dunbar, Angelo, Laura S, Kurzrock, Razelle
Format Journal Article
LanguageEnglish
Published United States Impact Journals LLC 15.01.2014
Subjects
Animals
Genes, Neurofibromatosis 2
Germ-Line Mutation
Humans
Nervous System Neoplasms - genetics
Neurofibromatosis 2 - genetics
Review
Signal Transduction
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Summary:Inactivating germline mutations in the tumor suppressor gene NF2 cause the hereditary syndrome neurofibromatosis 2, which is characterized by the development of neoplasms of the nervous system, most notably bilateral vestibular schwannoma. Somatic NF2 mutations have also been reported in a variety of cancers, but interestingly these mutations do not cause the same tumors that are common in hereditary neurofibromatosis 2, even though the same gene is involved and there is overlap in the site of mutations. This review highlights cancers in which somatic NF2 mutations have been found, the cell signaling pathways involving NF2/merlin, current clinical trials treating neurofibromatosis 2 patients, and preclinical findings that promise to lead to new targeted therapies for both cancers harboring NF2 mutations and neurofibromatosis 2 patients.
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ISSN:1949-2553
1949-2553
DOI:10.18632/oncotarget.1557