Merlin is the protein product of the NF2tumor suppressor gene. Germline mutations of the NF2 predispose affected individuals to the development of neurofibromatosis type 2, an autosomal dominant disorder that affects 1 in 33,000–40,000 people. Development of bilateral vestibular schwannomas is a hallmark of this NF2 syndrome; additional tumor types include schwannomas of other cranial, spinal, and cutaneous nerves, cranial and spinal meningiomas, as well as ependymomas and gliomas of the central nervous system. Biallelic inactivation of NF2 is also uniformly observed in sporadic vestibular schwannomas and in a large percentage of sporadic meningiomas. In addition, somatic genetic changes resulting in biallelic inactivation of NF2occur in about 50 % of malignant pleural mesotheliomas, which are mesodermally derived tumors unrelated to those found in the NF2 syndrome. Development of bilateral vestibular schwannomas is a hallmark of the...
KeywordsFocal Adhesion Kinase Malignant Pleural Mesothelioma Malignant Mesothelioma Hippo Signaling Spinal Meningioma
- Li W, et al (2010) Merlin/NF2 suppresses tumorigenesis by inhibiting the E3 ubiquitin ligase CRL4(DCAF1) in the nucleus. Cell 140:477–490Google Scholar
- McClatchey AI, Giovannini M (2005) Membrane organization and tumorigenesis – the NF2 tumor suppressor, Merlin. Genes Dev 19:2265–2277Google Scholar
- Petrilli AM, Fernandez-Valle C (2016) Role of Merlin/NF2 inactivation in tumor biology. Oncogene 35: 537–548Google Scholar
- Shapiro IM, et al (2014) Merlin deficiency predicts FAK inhibitor sensitivity: a synthetic lethal relationship. Sci Transl Med 6: 237ra68Google Scholar
- Zhang N, et al (2010) The Merlin/NF2 tumor suppressor functions through the YAP oncoprotein to regulate tissue homeostasis in mammals. Dev Cell 19:27–38Google Scholar