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Review

The Role of Merlin/NF2 Loss in Meningioma Biology

1
Department of Neurosurgery, Baylor College of Medicine, Houston, TX 77030, USA
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Department of Otolaryngology-Head and Neck Surgery, Baylor College of Medicine, Houston, TX 77030, USA
3
Jan and Dan Duncan Neurological Research Institute, Texas Children’s Hospital, Houston, TX 77030, USA
4
Department of Molecular and Human Genetics, Baylor College of Medicine, Houston, TX 77030, USA
*
Author to whom correspondence should be addressed.
Cancers 2019, 11(11), 1633; https://doi.org/10.3390/cancers11111633
Received: 13 September 2019 / Revised: 17 October 2019 / Accepted: 19 October 2019 / Published: 24 October 2019
(This article belongs to the Special Issue New Insights into Neurofibromatosis)
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. View Full-Text
Keywords: meningioma; merlin; neurofibromin 2; next-generation sequencing; DREAM complex meningioma; merlin; neurofibromin 2; next-generation sequencing; DREAM complex
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MDPI and ACS Style

Lee, S.; Karas, P.J.; Hadley, C.C.; Bayley V, J.C.; Khan, A.B.; Jalali, A.; Sweeney, A.D.; Klisch, T.J.; Patel, A.J. The Role of Merlin/NF2 Loss in Meningioma Biology. Cancers 2019, 11, 1633. https://doi.org/10.3390/cancers11111633

AMA Style

Lee S, Karas PJ, Hadley CC, Bayley V JC, Khan AB, Jalali A, Sweeney AD, Klisch TJ, Patel AJ. The Role of Merlin/NF2 Loss in Meningioma Biology. Cancers. 2019; 11(11):1633. https://doi.org/10.3390/cancers11111633

Chicago/Turabian Style

Lee, Sungho, Patrick J. Karas, Caroline C. Hadley, James C. Bayley V, A. B. Khan, Ali Jalali, Alex D. Sweeney, Tiemo J. Klisch, and Akash J. Patel 2019. "The Role of Merlin/NF2 Loss in Meningioma Biology" Cancers 11, no. 11: 1633. https://doi.org/10.3390/cancers11111633

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