The role of merlin/NF2 loss in meningioma biology

Sungho Lee, Patrick J. Karas, Caroline C. Hadley, James C. Bayley V., A. Basit Khan, Ali Jalali, Alex D. Sweeney, Tiemo J. Klisch, Akash J. Patel

Research output: Contribution to journalReview articlepeer-review

41 Scopus citations

Abstract

Mutations in the neurofibromin 2 (NF2) gene were among the first genetic alterations implicated in meningioma tumorigenesis, based on analysis of neurofibromatosis type 2 (NF2) patients who not only develop vestibular schwannomas but later have a high incidence of meningiomas. The NF2 gene product, merlin, is a tumor suppressor that is thought to link the actin cytoskeleton with plasma membrane proteins and mediate contact-dependent inhibition of proliferation. However, the early recognition of the crucial role of NF2 mutations in the pathogenesis of the majority of meningiomas has not yet translated into useful clinical insights, due to the complexity of merlin’s many interacting partners and signaling pathways. Next-generation sequencing studies and increasingly sophisticated NF2-deletion-based in vitro and in vivo models have helped elucidate the consequences of merlin loss in meningioma pathogenesis. In this review, we seek to summarize recent findings and provide future directions toward potential therapeutics for this tumor.

Original languageEnglish (US)
Article number1633
JournalCancers
Volume11
Issue number11
DOIs
StatePublished - Nov 2019
Externally publishedYes

Keywords

  • DREAM complex
  • Meningioma
  • Merlin
  • Neurofibromin 2
  • Next-generation sequencing

ASJC Scopus subject areas

  • Oncology
  • Cancer Research

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