{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,22]],"date-time":"2026-07-22T03:47:45Z","timestamp":1784692065834,"version":"3.55.0"},"publisher-location":"New York, NY","reference-count":78,"publisher":"Springer US","isbn-type":[{"value":"9781461406525","type":"print"},{"value":"9781461406532","type":"electronic"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2012]]},"DOI":"10.1007\/978-1-4614-0653-2_20","type":"book-chapter","created":{"date-parts":[[2012,3,12]],"date-time":"2012-03-12T08:35:13Z","timestamp":1331541313000},"page":"266-277","source":"Crossref","is-referenced-by-count":29,"title":["Neurofibromatoses"],"prefix":"10.1007","author":[{"given":"Erik J.","family":"Uhlmann","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Scott R.","family":"Plotkin","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","reference":[{"key":"20_CR1","first-page":"393","volume":"18","author":"JH Wishart","year":"1822","unstructured":"Wishart JH. Case of tumours in the skull, dura mater and brain. Edinburgh Med Surg J 1822; 18:393\u2013397.","journal-title":"Edinburgh Med Surg J"},{"key":"20_CR2","volume-title":"Uber die multiplen Fibrome der Haut und ihre Beziehung zu multiplen Neuromen","author":"F Recklinghausen von","year":"1882","unstructured":"von Recklinghausen F. Uber die multiplen Fibrome der Haut und ihre Beziehung zu multiplen Neuromen. Berlin, Germany: August Hirschwald; 1882."},{"key":"20_CR3","first-page":"259","volume":"29","author":"T Crump","year":"1981","unstructured":"Crump T. Translation of case reports in Ueber die multiplen Fibrome der Haut und ihre Beziehung zu den multiplen Neuromen by F. v. Recklinghausen. Adv Neurol 1981; 29:259\u2013275.","journal-title":"Adv Neurol"},{"key":"20_CR4","volume-title":"Neurofibromatosis: Phenotype, Natural History and Pathogenesis","author":"JM Friedman","year":"1999","unstructured":"Friedman JM, Gutmann DH, MacCollin M et al. Neurofibromatosis: Phenotype, Natural History and Pathogenesis, 3rd edition, Baltimore, Johns Hopkins Press; 1999.","edition":"3"},{"key":"20_CR5","first-page":"603","volume":"304","author":"DG Evans","year":"1992","unstructured":"Evans DG, Huson SM, Donnai D et al. A clinical study of type 2 neurofibromatosis. Q J Med 1992; 304:603\u2013618.","journal-title":"Q J Med"},{"key":"20_CR6","doi-asserted-by":"crossref","first-page":"71","DOI":"10.1212\/WNL.54.1.71","volume":"54","author":"J Antinheimo","year":"2000","unstructured":"Antinheimo J, Sankila R, Carpen O et al. Population-based analysis of sporadic and type 2 neurofibromatosis-associated meningiomas and schwannomas. Neurology 2000; 54:71\u201376.","journal-title":"Neurology"},{"key":"20_CR7","first-page":"653","volume":"15","author":"M Niimura","year":"1973","unstructured":"Niimura M. Neurofibromatosis. Rinsho Derma 1973; 15:653\u2013663.","journal-title":"Rinsho Derma"},{"issue":"12","key":"20_CR8","doi-asserted-by":"crossref","first-page":"1968","DOI":"10.1212\/01.WNL.0000070184.08740.E0","volume":"60","author":"M MacCollin","year":"2003","unstructured":"MacCollin M, Willett C, Heinrich B et al. Familial schwannomatosis: exclusion of the NF2 locus as the germline event. Neurology 2003; 60(12):1968\u20131974.","journal-title":"Neurology"},{"issue":"2","key":"20_CR9","doi-asserted-by":"publisher","first-page":"82","DOI":"10.1136\/jnnp.2010.206532","volume":"10","author":"RE Ferner","year":"2010","unstructured":"Ferner RE. The neurofibromatoses. Pract Neurol 2010; 10(2):82\u201393.","journal-title":"Pract Neurol"},{"issue":"18","key":"20_CR10","doi-asserted-by":"publisher","first-page":"1283","DOI":"10.1056\/NEJM199105023241812","volume":"324","author":"VM Riccardi","year":"1991","unstructured":"Riccardi VM. Neurofibromatosis: past, present and future. N Engl J Med 1991; 324(18):1283\u20131285.","journal-title":"N Engl J Med"},{"key":"20_CR11","doi-asserted-by":"publisher","first-page":"327","DOI":"10.1002\/ajmg.a.33139","volume":"152A","author":"DG Evans","year":"2010","unstructured":"Evans DG, Howard E, Giblin C et al. Birth incidence and prevalence of tumour prone syndromes: estimates from a UK genetic family register service. Am J Med Genet A 2010; 152A:327\u2013332.","journal-title":"Am J Med Genet A"},{"key":"20_CR12","doi-asserted-by":"publisher","first-page":"5","DOI":"10.1080\/02688690500081206","volume":"19","author":"DG Evans","year":"2005","unstructured":"Evans DG, Baser ME, O'Reilly B et al. Management of the patient and family with neurofibromatosis 2: a consensus conference statement. Br J Neurosurg 2005; 19:5\u201312.","journal-title":"Br J Neurosurg"},{"key":"20_CR13","doi-asserted-by":"publisher","first-page":"340","DOI":"10.1016\/S1474-4422(07)70075-3","volume":"6","author":"RE Ferner","year":"2007","unstructured":"Ferner RE. Neurofibromatosis 1 and neurofibromatosis 2: a twenty first century perspective. Lancet Neurol 2007; 6:340\u2013351.","journal-title":"Lancet Neurol"},{"key":"20_CR14","doi-asserted-by":"publisher","first-page":"223","DOI":"10.3171\/jns.2002.96.2.0223","volume":"96","author":"VF Mautner","year":"2002","unstructured":"Mautner VF, Baser ME, Thakkar SD et al. Vestibular schwannoma growth in patients with neurofibromatosis type 2: a longitudinal study. J Neurosurg 2002; 96:223\u2013228.","journal-title":"J Neurosurg"},{"key":"20_CR15","doi-asserted-by":"publisher","first-page":"353","DOI":"10.1016\/j.surneu.2003.11.020","volume":"62","author":"JH Huang","year":"2004","unstructured":"Huang JH, Simon SL, Nagpal S et al. Management of patients with schwannomatosis: report of six cases and review of the literature. Surg Neurol 2004; 62:353\u2013361.","journal-title":"Surg Neurol"},{"key":"20_CR16","doi-asserted-by":"crossref","first-page":"1072","DOI":"10.1212\/WNL.46.4.1072","volume":"46","author":"M MacCollin","year":"1996","unstructured":"MacCollin M, Woodfin W, Kronn D et al. Schwannomatosis-a clinical and pathologic study. Neurology 1996; 46:1072\u20131079.","journal-title":"Neurology"},{"key":"20_CR17","doi-asserted-by":"publisher","first-page":"228","DOI":"10.1159\/000245948","volume":"195","author":"P Wolkenstein","year":"1997","unstructured":"Wolkenstein P, Benchikhi H, Zeller J et al. Schwannomatosis: a clinical entity distinct from neurofibromatosis type 2. Dermatology 1997; 195:228\u2013231.","journal-title":"Dermatology"},{"issue":"2","key":"20_CR18","doi-asserted-by":"publisher","first-page":"231","DOI":"10.1086\/422700","volume":"75","author":"ME Baser","year":"2004","unstructured":"Baser ME, Kuramoto L, Joe H et al. Genotype-phenotype correlations for nervous system tumors in neurofibromatosis 2: a population-based study. Am J Hum Genet 2004; 75(2):231\u2013239.","journal-title":"Am J Hum Genet"},{"key":"20_CR19","doi-asserted-by":"publisher","first-page":"931","DOI":"10.1016\/0888-7543(91)90017-9","volume":"4","author":"DA Marchuk","year":"1991","unstructured":"Marchuk DA, Saulino AM, Tavakkol R et al. cDNA cloning of the type 1 neurofibromatosis gene: complete sequence of the NF1 gene product. Genomics 1991; 4:931\u2013940.","journal-title":"Genomics"},{"issue":"6429","key":"20_CR20","doi-asserted-by":"publisher","first-page":"515","DOI":"10.1038\/363515a0","volume":"363","author":"GA Rouleau","year":"1993","unstructured":"Rouleau GA, Merel P, Lutchman M et al. Alteration in a new gene encoding a putative membrane-organizing protein causes neuro-fibromatosis type 2. Nature 1993; 363(6429):515\u2013521.","journal-title":"Nature"},{"issue":"9","key":"20_CR21","doi-asserted-by":"publisher","first-page":"1361","DOI":"10.1093\/hmg\/2.9.1361","volume":"2","author":"YG Xie","year":"1993","unstructured":"Xie YG, Han FY, Peyrard M et al. Cloning of a novel, anonymous gene from a megabase-range YAC and cosmid contig in the neurofibromatosis type 2\/meningioma region on human chromosome 22q12. Hum Mol Genet 1993; 2(9):1361\u20131368.","journal-title":"Hum Mol Genet"},{"key":"20_CR22","doi-asserted-by":"publisher","first-page":"791","DOI":"10.1016\/0092-8674(93)90406-G","volume":"72","author":"JA Trofatter","year":"1993","unstructured":"Trofatter JA, MacCollin MM, Rutter JL et al. A novel moesin-, ezrin-, radixin-like gene is a candidate for the neurofibromatosis 2 tumor suppressor. Cell 1993; 72:791\u2013800.","journal-title":"Cell"},{"key":"20_CR23","doi-asserted-by":"publisher","first-page":"820","DOI":"10.1073\/pnas.68.4.820","volume":"68","author":"AG Knudson","year":"1971","unstructured":"Knudson AG. Mutation and cancer: statistical study of retinoblastoma. Proc Natl Acad Sci USA 1971; 68:820\u2013823.","journal-title":"Proc Natl Acad Sci USA"},{"key":"20_CR24","first-page":"74","volume":"59","author":"JA Biegel","year":"1999","unstructured":"Biegel JA, Zhou JY, Rorke LB et al. Germ-line and acquired mutations of INI1 in atypical teratoid and rhabdoid tumors. Cancer Res 1999; 59:74\u201379.","journal-title":"Cancer Res"},{"key":"20_CR25","doi-asserted-by":"publisher","first-page":"1342","DOI":"10.1086\/302639","volume":"65","author":"N Sevenet","year":"1999","unstructured":"Sevenet N, Sheridan E, Amram D et al. Constitutional mutations of the hSNF5\/INI1 gene predispose to a variety of cancers. Am J Hum Genet 1999a; 65:1342\u20131348.","journal-title":"Am J Hum Genet"},{"key":"20_CR26","doi-asserted-by":"publisher","first-page":"257","DOI":"10.1023\/A:1024345221792","volume":"63","author":"H Fujisawa","year":"2003","unstructured":"Fujisawa H, Takabatake Y, Fukusato T et al. Molecular analysis of the rhabdoid predisposition syndrome in a child: a novel germline hSNF5\/INI1 mutation and absence of c-myc amplification. J Neurooncol 2003; 63:257\u2013262.","journal-title":"J Neurooncol"},{"key":"20_CR27","doi-asserted-by":"publisher","first-page":"1317","DOI":"10.1001\/archneur.60.9.1317","volume":"60","author":"DL Kaufman","year":"2003","unstructured":"Kaufman DL, Heinrich BS, Willett C et al. Somatic instability of the NF2 gene in schwannomatosis. Arch Neurol 2003; 60:1317\u20131320.","journal-title":"Arch Neurol"},{"issue":"1","key":"20_CR28","doi-asserted-by":"publisher","first-page":"193","DOI":"10.1016\/0092-8674(90)90253-B","volume":"62","author":"RM Cawthon","year":"1990","unstructured":"Cawthon RM, Weiss R, Xu GF et al. A major segment of the neurofibromatosis type 1 gene: cDNA sequence, genomic structure and point mutations. Cell 1990; 62(1):193\u2013201.","journal-title":"Cell"},{"issue":"1","key":"20_CR29","doi-asserted-by":"publisher","first-page":"187","DOI":"10.1016\/0092-8674(90)90252-A","volume":"62","author":"D Viskochil","year":"1990","unstructured":"Viskochil D, Buchberg AM, Xu G et al. Deletions and a translocation interrupt a cloned gene at the neurofibromatosis type 1 locus. Cell 1990; 62(1):187\u2013192.","journal-title":"Cell"},{"issue":"4965","key":"20_CR30","doi-asserted-by":"publisher","first-page":"181","DOI":"10.1126\/science.2134734","volume":"249","author":"MR Wallace","year":"1990","unstructured":"Wallace MR, Marchuk DA, Andersen LB et al. Type 1 neurofibromatosis gene: identification of a large transcript disrupted in three NF1 patients. Science 1990; 249(4965):181\u2013186.","journal-title":"Science"},{"key":"20_CR31","doi-asserted-by":"publisher","first-page":"663","DOI":"10.1038\/356663a0","volume":"356","author":"G Bollag","year":"1992","unstructured":"Bollag G, McCormick F. Ras regulation. NF is enough of GAP. Nature 1992; 356:663\u2013664.","journal-title":"Nature"},{"key":"20_CR32","doi-asserted-by":"publisher","first-page":"283","DOI":"10.1002\/(SICI)1098-2264(199903)24:3<283::AID-GCC15>3.0.CO;2-K","volume":"24","author":"L Kluwe","year":"1999","unstructured":"Kluwe L, Friedrich R, Mautner VF. Loss of NF1 allele in Schwann cells but not in fibroblasts derived from an NF1-associated neurofibroma. Genes Chromosomes Cancer 1999; 24:283\u2013285.","journal-title":"Genes Chromosomes Cancer"},{"key":"20_CR33","doi-asserted-by":"publisher","first-page":"1059","DOI":"10.1093\/hmg\/9.7.1059","volume":"9","author":"JL Rutkowski","year":"2000","unstructured":"Rutkowski JL, Wu K, Gutmann DH et al. Genetic and cellular defects contributing to benign tumor formation in neurofibromatosis type 1. Hum Mol Genet 2000; 9:1059\u20131066.","journal-title":"Hum Mol Genet"},{"key":"20_CR34","doi-asserted-by":"publisher","first-page":"122","DOI":"10.1038\/ng0293-122","volume":"3","author":"E Legius","year":"1993","unstructured":"Legius E, Marchuk DA, Collins FS et al. Somatic deletion of the neurofibromatosis type 1 gene in a neurofibrosarcoma supports a tumour suppressor gene hypothesis. Nat Genet 1993; 3:122\u2013126.","journal-title":"Nat Genet"},{"issue":"16","key":"20_CR35","doi-asserted-by":"publisher","first-page":"5579","DOI":"10.1523\/JNEUROSCI.3994-09.2010","volume":"30","author":"JA Brown","year":"2010","unstructured":"Brown JA, Gianino SM, Gutmann DH. Defective cAMP generation underlies the sensitivity of CNS neurons to neurofibromatosis-1 heterozygosity. J Neurosci 2010; 30(16):5579\u20135589.","journal-title":"J Neurosci"},{"issue":"14","key":"20_CR36","doi-asserted-by":"publisher","first-page":"5717","DOI":"10.1158\/0008-5472.CAN-09-3769","volume":"70","author":"NM Warrington","year":"2010","unstructured":"Warrington NM, Gianino SM, Jackson E et al. Cyclic AMP suppression is sufficient to induce gliomagenesis in a mouse model of neurofibromatosis-1. Cancer Res 2010; 70(14):5717\u20135727.","journal-title":"Cancer Res"},{"issue":"4","key":"20_CR37","doi-asserted-by":"publisher","first-page":"443","DOI":"10.1016\/j.stem.2007.07.008","volume":"1","author":"B Hegedus","year":"2007","unstructured":"Hegedus B, Dasgupta B, Shin JE et al. Neurofibromatosis-1 regulates neuronal and glial cell differentiation from neuroglial progenitors in vivo by both cAMP-and Ras-dependent mechanisms. Cell Stem Cell 2007; 1(4):443\u2013457.","journal-title":"Cell Stem Cell"},{"issue":"20","key":"20_CR38","doi-asserted-by":"publisher","first-page":"2317","DOI":"10.1101\/gad.1957110","volume":"24","author":"DY Lee","year":"2010","unstructured":"Lee DY, Yeh TH, Emnett RJ et al. Neurofibromatosis-1 regulates neuroglial progenitor proliferation and glial differentiation in a brain region-specific manner. Genes Dev 2010; 24(20):2317\u20132329.","journal-title":"Genes Dev"},{"issue":"23","key":"20_CR39","doi-asserted-by":"publisher","first-page":"4643","DOI":"10.1093\/hmg\/ddq395","volume":"19","author":"JS Lee","year":"2010","unstructured":"Lee JS, Padmanabhan A, Shin J et al. Oligodendrocyte progenitor cell numbers and migration are regulated by the zebrafish orthologs of the NF1 tumor suppressor gene. Hum Mol Genet 2010; 19(23):4643\u20134653.","journal-title":"Hum Mol Genet"},{"issue":"4","key":"20_CR40","doi-asserted-by":"publisher","first-page":"287","DOI":"10.1097\/MOH.0b013e328339511b","volume":"17","author":"K Staser","year":"2010","unstructured":"Staser K, Yang FC, Clapp DW. Plexiform neurofibroma genesis: questions of Nf1 gene dose and hyperactive mast cells. Curr Opin Hematol 2010; 17(4):287\u2013293.","journal-title":"Curr Opin Hematol"},{"key":"20_CR41","doi-asserted-by":"crossref","unstructured":"Chai G, Liu N, Ma J et al. MicroRNA-10b regulates tumorigenesis in neurofibromatosis type 1. Cancer Sci 2010.","DOI":"10.1111\/j.1349-7006.2010.01616.x"},{"issue":"25","key":"20_CR42","doi-asserted-by":"publisher","first-page":"3167","DOI":"10.1093\/hmg\/11.25.3167","volume":"11","author":"CX Sun","year":"2002","unstructured":"Sun CX, Haipek C, Scoles DR et al. Functional analysis of the relationship between the neurofibromatosis 2 tumor suppressor and its binding partner, hepatocyte growth factor-regulated tyrosine kinase substrate. Hum Mol Genet 2002; 11(25):3167\u20133178.","journal-title":"Hum Mol Genet"},{"issue":"6","key":"20_CR43","doi-asserted-by":"publisher","first-page":"462","DOI":"10.1038\/nrn1407","volume":"5","author":"V Ramesh","year":"2004","unstructured":"Ramesh V. Merlin and the ERM proteins in Schwann cells, neurons and growth cones. Nat Rev Neurosci 2004; 5(6):462\u2013470.","journal-title":"Nat Rev Neurosci"},{"issue":"11","key":"20_CR44","first-page":"2270","volume":"55","author":"M Lutchman","year":"1995","unstructured":"Lutchman M, Rouleau GA. The neurofibromatosis type 2 gene product, schwannomin, suppresses growth of NIH 3T3 cells. Cancer Res 1995; 55(11):2270\u20132274.","journal-title":"Cancer Res"},{"issue":"10","key":"20_CR45","doi-asserted-by":"publisher","first-page":"1253","DOI":"10.1101\/gad.11.10.1253","volume":"11","author":"AI McClatchey","year":"1997","unstructured":"McClatchey AI, Saotome I, Ramesh V et al. Genes Dev 1997; 11(10):1253\u20131265. The Nf2 tumor suppressor gene product is essential for extraembryonic development immediately prior to gastrulation. Genes Dev 1997; 11(10):1253\u20131265.","journal-title":"Genes Dev"},{"issue":"20","key":"20_CR46","doi-asserted-by":"publisher","first-page":"2505","DOI":"10.1038\/sj.onc.1201418","volume":"15","author":"L Sherman","year":"1997","unstructured":"Sherman L, Xu HM, Geist RT et al. Interdomain binding mediates tumor growth suppression by the NF2 gene product. Oncogene 1997; 15(20):2505\u20132509.","journal-title":"Oncogene"},{"issue":"1","key":"20_CR47","doi-asserted-by":"publisher","first-page":"85","DOI":"10.3171\/jns.1999.91.1.0085","volume":"91","author":"K Ikeda","year":"1999","unstructured":"Ikeda K, Saeki Y, Gonzalez-Agosti C et al. Inhibition of NF2-negative and NF2-positive primary human meningioma cell proliferation by overexpression of merlin due to vector-mediated gene transfer. J Neurosurg 1999; 91(1):85\u201392.","journal-title":"J Neurosurg"},{"issue":"6","key":"20_CR48","first-page":"1493","volume":"23","author":"JT Fraenzer","year":"2003","unstructured":"Fraenzer JT, Pan H, Minimo L Jr et al. Overexpression of the NF2 gene inhibits schwannoma cell proliferation through promoting PDGFR degradation. Int J Oncol 2003; 23(6):1493\u20131500.","journal-title":"Int J Oncol"},{"issue":"6","key":"20_CR49","doi-asserted-by":"publisher","first-page":"2384","DOI":"10.1128\/MCB.25.6.2384-2394.2005","volume":"25","author":"GH Xiao","year":"2005","unstructured":"Xiao GH, Gallagher R, Shetler J et al. The NF2 tumor suppressor gene product, merlin, inhibits cell proliferation and cell cycle progression by repressing cyclin D1 expression. Mol Cell Biol 2005; 25(6):2384\u20132394.","journal-title":"Mol Cell Biol"},{"issue":"1","key":"20_CR50","doi-asserted-by":"publisher","first-page":"63","DOI":"10.1016\/S1534-5807(01)00009-0","volume":"1","author":"RJ Shaw","year":"2001","unstructured":"Shaw RJ, Paez JG, Curto M et al. The Nf2 tumor suppressor, merlin, functions in Rac-dependent signaling. Dev Cell 2001; 1(1):63\u201372.","journal-title":"Dev Cell"},{"key":"20_CR51","doi-asserted-by":"publisher","first-page":"841","DOI":"10.1016\/S1097-2765(03)00382-4","volume":"12","author":"JL Kissil","year":"2003","unstructured":"Kissil JL, Wilker EW, Johnson KC et al. Merlin, the product of the Nf2 tumor suppressor gene, is an inhibitor of the p21-activated kinase, Pak1. Mol Cell 2003; 12:841\u2013849.","journal-title":"Mol Cell"},{"issue":"12","key":"20_CR52","doi-asserted-by":"publisher","first-page":"4463","DOI":"10.1073\/pnas.0510651103","volume":"103","author":"DN Chadee","year":"2006","unstructured":"Chadee DN, Xu D, Hung G et al. Mixed-lineage kinase 3 regulates B-Raf through maintenance of the B-Raf\/Raf-1 complex and inhibition by the NF2 tumor suppressor protein. Proc Natl Acad Sci USA 2006; 103(12):4463\u20134468.","journal-title":"Proc Natl Acad Sci USA"},{"issue":"2","key":"20_CR53","doi-asserted-by":"publisher","first-page":"520","DOI":"10.1158\/0008-5472.CAN-06-1608","volume":"67","author":"H Morrison","year":"2007","unstructured":"Morrison H, Sperka T, Manent J et al. Merlin\/neurofibromatosis type 2 suppresses growth by inhibiting the activation of Ras and Rac. Cancer Res 2007; 67(2):520\u2013527.","journal-title":"Cancer Res"},{"issue":"7","key":"20_CR54","doi-asserted-by":"publisher","first-page":"1253","DOI":"10.1016\/j.cell.2006.05.030","volume":"125","author":"LZ Zender","year":"2006","unstructured":"Z Zender L, Spector MS, Xue W et al. Identification and validation of oncogenes in liver cancer using an integrative oncogenomic approach. Cell 2006; 125(7):1253\u20131267.","journal-title":"Cell"},{"issue":"6","key":"20_CR55","doi-asserted-by":"publisher","first-page":"1120","DOI":"10.1016\/j.cell.2007.07.019","volume":"130","author":"J Dong","year":"2007","unstructured":"Dong J, Feldmann G, Huang J et al. Elucidation of a universal size-control mechanism in Drosophila and mammals. Cell 2007; 130(6):1120\u20131133.","journal-title":"Cell"},{"issue":"5","key":"20_CR56","doi-asserted-by":"publisher","first-page":"425","DOI":"10.1016\/j.ccr.2009.09.026","volume":"16","author":"D Zhou","year":"2009","unstructured":"Zhou D, Conrad C, Xia F et al. Mst1 and Mst2 maintain hepatocyte quiescence and suppress hepatocellular carcinoma development through inactivation of the Yap1 oncogene. Cancer Cell 2009; 16(5):425\u2013438.","journal-title":"Cancer Cell"},{"issue":"1","key":"20_CR57","doi-asserted-by":"publisher","first-page":"27","DOI":"10.1016\/j.devcel.2010.06.015","volume":"19","author":"N Zhang","year":"2010","unstructured":"Zhang N, Bai H, David KK et al. The Merlin\/NF2 tumor suppressor functions through the YAP oncoprotein to regulate tissue homeostasis in mammals. Dev Cell 2010; 19(1):27\u201338.","journal-title":"Dev Cell"},{"issue":"4","key":"20_CR58","doi-asserted-by":"publisher","first-page":"1437","DOI":"10.1073\/pnas.0911427107","volume":"107","author":"L Lu","year":"2010","unstructured":"Lu L, Li Y, Kim SM et al. Hippo signaling is a potent in vivo growth and tumor suppressor pathway in the mammalian liver. Proc Natl Acad Sci USA 2010;107(4):1437\u20131442.","journal-title":"Proc Natl Acad Sci USA"},{"issue":"4","key":"20_CR59","doi-asserted-by":"publisher","first-page":"1431","DOI":"10.1073\/pnas.0911409107","volume":"107","author":"H Song","year":"2010","unstructured":"Song H, Mak KK, Topol L et al. Mammalian Mst1 and Mst2 kinases play essential roles in organ size control and tumor suppression. Proc Natl Acad Sci USA 2010; 107(4):1431\u20131436.","journal-title":"Proc Natl Acad Sci USA"},{"key":"20_CR60","doi-asserted-by":"crossref","unstructured":"Zhan Y, Modi N, Stewart AM et al. Regulation of mixed lineage kinase 3 is required for Neurofibromatosis-2-mediated growth suppression in human cancer. Oncogene 2010.","DOI":"10.1038\/onc.2010.453"},{"issue":"16","key":"20_CR61","doi-asserted-by":"publisher","first-page":"1718","DOI":"10.1101\/gad.1938710","volume":"24","author":"S Benhamouche","year":"2010","unstructured":"Benhamouche S, Curto M, Saotome I et al. Nf2\/Merlin controls progenitor homeostasis and tumorigenesis in the liver. Genes Dev 2010; 24(16):1718\u20131730.","journal-title":"Genes Dev"},{"issue":"4","key":"20_CR62","doi-asserted-by":"publisher","first-page":"1274","DOI":"10.1128\/MCB.01139-07","volume":"28","author":"BK Cole","year":"2008","unstructured":"Cole BK, Curto M, Chan AW et al. Localization to the cortical cytoskeleton is necessary for Nf2\/ merlin-dependent epidermal growth factor receptor silencing. Mol Cell Biol 2008; 28(4):1274\u20131284.","journal-title":"Mol Cell Biol"},{"issue":"15","key":"20_CR63","doi-asserted-by":"publisher","first-page":"4250","DOI":"10.1128\/MCB.01581-08","volume":"29","author":"MF James","year":"2009","unstructured":"James MF, Han S, Polizzano C et al. NF2\/merlin is a novel negative regulator of mTOR complex 1 and activation of mTORC1 is associated with meningioma and schwannoma growth. Mol Cell Biol 2009; 29(15):4250\u20134261.","journal-title":"Mol Cell Biol"},{"issue":"4","key":"20_CR64","doi-asserted-by":"publisher","first-page":"477","DOI":"10.1016\/j.cell.2010.01.029","volume":"140","author":"W Li","year":"2010","unstructured":"Li W, You L, Cooper J et al. Merlin\/NF2 suppresses tumorigenesis by inhibiting the E3 ubiquitin ligase CRL4 (DCAF1) in the nucleus. Cell 2010; 140(4):477\u2013490.","journal-title":"Cell"},{"issue":"4","key":"20_CR65","doi-asserted-by":"publisher","first-page":"805","DOI":"10.1086\/513207","volume":"80","author":"TJ Hulsebos","year":"2007","unstructured":"Hulsebos TJ, Plomp AS, Wolterman RA et al. Am J Hum Genet 2007; 80(4):805\u2013810.","journal-title":"Am J Hum Genet"},{"issue":"16","key":"20_CR66","doi-asserted-by":"publisher","first-page":"5975","DOI":"10.1128\/MCB.22.16.5975-5988.2002","volume":"22","author":"ZK Zhang","year":"2002","unstructured":"Zhang ZK, Davies KP, Allen J et al. Cell cycle arrest and repression of cyclin D1 transcription by INI1\/hSNF5. Mol Cell Biol 2002; 22(16):5975\u20138859.","journal-title":"Mol Cell Biol"},{"issue":"34","key":"20_CR67","doi-asserted-by":"publisher","first-page":"12129","DOI":"10.1073\/pnas.0505300102","volume":"102","author":"M Tsikitis","year":"2005","unstructured":"Tsikitis M, Zhang Z, Edelman W et al. Genetic ablation of Cyclin D1 abrogates genesis of rhabdoid tumors resulting from Ini1 loss. Proc Natl Acad Sci USA 2005; 102(34):12129\u201312134.","journal-title":"Proc Natl Acad Sci USA"},{"issue":"2","key":"20_CR68","doi-asserted-by":"publisher","first-page":"117","DOI":"10.1007\/s11060-004-4276-4","volume":"73","author":"H Fujisawa","year":"2005","unstructured":"Fujisawa H, Misaki K, Takabatake Y et al. Cyclin D1 is overexpressed in atypical teratoid\/rhabdoid tumor with hSNF5\/INI1 gene inactivation. J Neurooncol 2005; 73(2):117\u2013124.","journal-title":"J Neurooncol"},{"issue":"11","key":"20_CR69","doi-asserted-by":"crossref","first-page":"1759","DOI":"10.1212\/01.WNL.0000035638.74084.F4","volume":"59","author":"ME Baser","year":"2002","unstructured":"Baser ME, Friedman JM, Wallace AJ et al. Evaluation of clinical diagnostic criteria for neurofibromatosis 2. Neurology 2002; 59(11):1759\u20131765.","journal-title":"Neurology"},{"issue":"5","key":"20_CR70","doi-asserted-by":"publisher","first-page":"730","DOI":"10.1212\/01.wnl.0000201190.89751.41","volume":"66","author":"ME Baser","year":"2006","unstructured":"Baser ME, Friedman JM, Evans DG. Increasing the specificity of diagnostic criteria for schwannomatosis. Neurology 2006; 66(5):730\u2013732.","journal-title":"Neurology"},{"issue":"11","key":"20_CR71","doi-asserted-by":"publisher","first-page":"1838","DOI":"10.1212\/01.WNL.0000163982.78900.AD","volume":"64","author":"M MacCollin","year":"2005","unstructured":"MacCollin M, Chiocca EA, Evans DG et al. Diagnostic criteria for schwannomatosis. Neurology 2005; 64(11):1838\u20131845.","journal-title":"Neurology"},{"issue":"6","key":"20_CR72","doi-asserted-by":"publisher","first-page":"1314","DOI":"10.1227\/01.neu.0000333303.79931.83","volume":"62","author":"GJ Harris","year":"2008","unstructured":"Harris GJ, Plotkin SR, Maccollin M et al. Three-dimensional volumetrics for tracking vestibular schwannoma growth in neurofibromatosis type II. Neurosurgery 2008; 62(6):1314\u20131319.","journal-title":"Neurosurgery"},{"key":"20_CR73","first-page":"167","volume":"64","author":"V Colletti","year":"2006","unstructured":"Colletti V. Auditory outcomes in tumor vs nontumor patients fitted with auditory brainstem implants. Adv Otorhinolaryngol 2006; 64:167\u2013185.","journal-title":"Adv Otorhinolaryngol"},{"issue":"4","key":"20_CR74","doi-asserted-by":"publisher","first-page":"358","DOI":"10.1056\/NEJMoa0902579","volume":"361","author":"SR Plotkin","year":"2009","unstructured":"Plotkin SR, Stemmer-Rachamimov AO, Barker FG 2nd et al. Hearing improvement after bevacizumab in patients with neurofibromatosis type 2. N Engl J Med 2009; 361(4):358\u2013367.","journal-title":"N Engl J Med"},{"issue":"4","key":"20_CR75","doi-asserted-by":"publisher","first-page":"715","DOI":"10.1086\/342716","volume":"71","author":"ME Baser","year":"2002","unstructured":"Baser ME, Friedman JM, Aeschliman D et al. Predictors of the risk of mortality in neurofibromatosis 2. Am J Hum Genet 2002; 71(4):715\u2013723.","journal-title":"Am J Hum Genet"},{"issue":"6","key":"20_CR76","doi-asserted-by":"publisher","first-page":"541","DOI":"10.1002\/1098-1004(200006)15:6<541::AID-HUMU6>3.0.CO;2-N","volume":"15","author":"LM Messiaen","year":"2000","unstructured":"Messiaen LM, Callens T, Mortier G et al. Exhaustive mutation analysis of the NF1 gene allows identi-fication of 95% of mutations and reveals a high frequency of unusual splicing defects. Hum Mutat 2000; 15(6):541\u2013555.","journal-title":"Hum Mutat"},{"issue":"5","key":"20_CR77","doi-asserted-by":"publisher","first-page":"381","DOI":"10.1093\/molehr\/gah170","volume":"11","author":"C Spits","year":"2005","unstructured":"Spits C, De Rycke M, Van Ranst N et al. Preimplantation genetic diagnosis for neurofibromatosis type 1. Mol Hum Reprod 2005; 11(5):381\u2013387.","journal-title":"Mol Hum Reprod"},{"issue":"6","key":"20_CR78","doi-asserted-by":"publisher","first-page":"459","DOI":"10.1136\/jmg.40.6.459","volume":"40","author":"A Moyhuddin","year":"2003","unstructured":"Moyhuddin A, Baser ME, Watson C et al. Somatic mosaicism in neurofibromatosis 2: prevalence and risk of disease transmission to offspring. J Med Genet 2003; 40(6):459\u2013463.","journal-title":"J Med Genet"}],"container-title":["Advances in Experimental Medicine and Biology","Neurodegenerative Diseases"],"original-title":[],"link":[{"URL":"http:\/\/link.springer.com\/content\/pdf\/10.1007\/978-1-4614-0653-2_20.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2021,5,1]],"date-time":"2021-05-01T04:41:28Z","timestamp":1619844088000},"score":1,"resource":{"primary":{"URL":"http:\/\/link.springer.com\/10.1007\/978-1-4614-0653-2_20"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2012]]},"ISBN":["9781461406525","9781461406532"],"references-count":78,"URL":"https:\/\/doi.org\/10.1007\/978-1-4614-0653-2_20","relation":{},"ISSN":["0065-2598"],"issn-type":[{"value":"0065-2598","type":"print"}],"subject":[],"published":{"date-parts":[[2012]]}}}