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Neurofibromatosis type 2 (NF2) is a monogenic dominantly inherited disease predisposing carriers to develop nervous system tumours. To identify the genetic defect, the region between two flanking polymorphic markers on chromosome 22 was cloned and several genes identified. One is the site of germ-line mutations in NF2 patients and of somatic mutations in(More)
Neurofibromatosis type 2 (NF2) is an autosomal dominant disease in which loss of function mutations of the NF2 gene lead to the development of schwannomas, meningiomas and juvenile cataracts. We studied the mouse NF2 homologue (Nf2) to determine its precise pattern of mRNA and protein expression. In situ hybridization showed that Nf2 is expressed in(More)
Neurofibromatosis 2 (NF2) protein (merlin; schwannomin) is a tumor suppressor involved in tumorigenesis of NF2-associated and sporadic schwannomas and meningiomas. The protein shares the domain structure of three homologous proteins: ezrin, radixin and moesin (ERM). ERM proteins function as membrane organizers and may act as linkers between plasma membrane(More)
Cancer is a multistep process that involves the activation of oncogenes and the inactivation of antioncogenes. Recently, a new putative tumor suppressor, the neurofibromatosis type 2 (NF2) gene, was mapped to chromosome 22, cloned, and found to encode for a new protein, merlin/schwannomin, a member of the band 4.1 family of proteins. Members of this family(More)
Neurofibromatosis type 2 (NF2) is an autosomal dominant disease which predisposes primarily to CNS tumors such as schwannomas (vestibular and spinal), meningiomas, ependymomas and juvenile posterior lenticular opacities. Allelic losses on chromosome 22q first suggested the existence of a tumor suppressor on this autosome in accordance with Knudson's 'two(More)
BACKGROUND Neurofibromatosis type 2 (NF2) is an autosomal dominant disease predisposing individuals to the risk of developing tumors of cranial and spinal nerves. The NF2 tumor suppressor protein, known as Merlin/Schwanomin, is a member of the protein 4.1 superfamily that function as links between the cytoskeleton and the plasma membrane. METHODS Upon(More)
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