Molecular biology of familial and sporadic vestibular schwannomas: Implications for novel therapeutics. A review

Michael E. Sughrue, Andrea H. Yeung, Martin J. Rutkowski, Steven W. Cheung, Andrew T. Parsa

Research output: Contribution to journalReview article

27 Scopus citations

Abstract

Vestibular schwannomas (VSs) are benign tumors arising from the sheath of cranial nerve VIII. The pathogenesis underlying most familial and sporadic VSs has been linked to a mutation in a single gene, the neurofibromin 2 (NF2) gene located on chromosome 22, band q11-13.1. In this review, the authors summarized what is known about the epidemiology of NF2 mutations and patients with VSs. The authors also discuss the function of the NF2 gene product, merlin, and describe the known and hypothetical effects of genetic mutations that lead to merlin dysfunction on a broad variety of cellular and histological end points. A better understanding of the molecular pathobiology of VSs may lead to novel therapeutics to augment current modalities of treatment while minimizing morbidity.

Original languageEnglish (US)
Pages (from-to)359-366
Number of pages8
JournalJournal of neurosurgery
Volume114
Issue number2
DOIs
StatePublished - Feb 2011

Keywords

  • Merlin
  • Neurofibromatosis type 2
  • Vestibular schwannoma

ASJC Scopus subject areas

  • Surgery
  • Clinical Neurology

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