{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,11,13]],"date-time":"2025-11-13T12:17:51Z","timestamp":1763036271084},"reference-count":14,"publisher":"Cambridge University Press (CUP)","issue":"5","license":[{"start":{"date-parts":[[2007,6,29]],"date-time":"2007-06-29T00:00:00Z","timestamp":1183075200000},"content-version":"unspecified","delay-in-days":4442,"URL":"https:\/\/www.cambridge.org\/core\/terms"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["J. Laryngol. Otol."],"published-print":{"date-parts":[[1995,5]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Vestibular schwannoma occurs both as a sporadic tumour and in the dominantly inherited familial cancer syndrome neurofibromatosis type 2 (NF2). The gene for NF2 has recently beenisolated on chromosome 22, and the demonstration of inactivating germline mutations in NF2 patients and NF2 associated tumours suggests that it acts as a tumour suppressor. The results of recent research in Cambridge suggest that somatic mutations of the NF2 tumour suppressor gene are a critical step in the pathogenesis of both familial and indeed non-familial unilateral sporadic vestibular schwannoma and that the mechanism of tumourigenesis complies with the \u2018two-hit\u2019 model. This paper represents a brief review of the current status of molecular biology in relation to vestibular schwannoma in particular and is discussed in relation to the molecular pathology of skull base tumours as a whole.<\/jats:p>","DOI":"10.1017\/s0022215100130245","type":"journal-article","created":{"date-parts":[[2007,11,30]],"date-time":"2007-11-30T10:15:32Z","timestamp":1196417732000},"page":"381-384","source":"Crossref","is-referenced-by-count":15,"title":["The molecular genetics of vestibular schwannoma"],"prefix":"10.1017","volume":"109","author":[{"given":"David A.","family":"Moffat","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Richard M.","family":"Irving","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"56","published-online":{"date-parts":[[2007,6,29]]},"reference":[{"key":"S0022215100130245_ref013","doi-asserted-by":"publisher","DOI":"10.1017\/S0022215100147292"},{"key":"S0022215100130245_ref008","first-page":"413","volume":"3","author":"Jacoby","year":"1994","journal-title":"Exon scanning for mutation of the NF2 gene in schwannomas Human Molecular Genetics"},{"key":"S0022215100130245_ref005","doi-asserted-by":"publisher","DOI":"10.1093\/hmg\/3.2.347"},{"key":"S0022215100130245_ref004","doi-asserted-by":"publisher","DOI":"10.1001\/archotol.1993.01880230066011"},{"key":"S0022215100130245_ref014","first-page":"1","volume":"72","author":"Troffater","year":"1993","journal-title":"A novel moesin-, ezrin-, radixin-like gene is a candidate for the neurofibromatosis type 2 tumour suppressor"},{"key":"S0022215100130245_ref002","doi-asserted-by":"publisher","DOI":"10.1136\/jmg.29.12.841"},{"key":"S0022215100130245_ref001","doi-asserted-by":"publisher","DOI":"10.1083\/jcb.108.3.921"},{"key":"S0022215100130245_ref006","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2273.1995.tb00003.x"},{"key":"S0022215100130245_ref010","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2273.1995.tb00018.x"},{"key":"S0022215100130245_ref012","doi-asserted-by":"publisher","DOI":"10.1038\/322644a0"},{"key":"S0022215100130245_ref009","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.68.4.820"},{"key":"S0022215100130245_ref011","doi-asserted-by":"publisher","DOI":"10.1038\/363515a0"},{"key":"S0022215100130245_ref003","first-page":"1335","article-title":"The ezrin-like family of tyrosine kinase substrates: receptor-specific pattern of tyrosine phophorylation and relationship to malignant transformation","volume":"8","author":"Fazioli","year":"1993","journal-title":"Oncogene"},{"key":"S0022215100130245_ref007","first-page":"191","volume-title":"The molecular pathology of skull base tumours In Recent Advances in Otolaryngology number 7","author":"Irving","year":"1995"}],"container-title":["The Journal of Laryngology &amp; Otology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.cambridge.org\/core\/services\/aop-cambridge-core\/content\/view\/S0022215100130245","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2019,5,13]],"date-time":"2019-05-13T22:45:03Z","timestamp":1557787503000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.cambridge.org\/core\/product\/identifier\/S0022215100130245\/type\/journal_article"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[1995,5]]},"references-count":14,"journal-issue":{"issue":"5","published-print":{"date-parts":[[1995,5]]}},"alternative-id":["S0022215100130245"],"URL":"https:\/\/doi.org\/10.1017\/s0022215100130245","relation":{},"ISSN":["0022-2151","1748-5460"],"issn-type":[{"value":"0022-2151","type":"print"},{"value":"1748-5460","type":"electronic"}],"subject":[],"published":{"date-parts":[[1995,5]]}}}