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© 2019 by the authors. Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (http://creativecommons.org/licenses/by/4.0/). Notwithstanding the ProQuest Terms and Conditions, you may use this content in accordance with the terms of the License.

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.

Details

Title
The Role of Merlin/NF2 Loss in Meningioma Biology
Author
Lee, Sungho 1 ; Karas, Patrick J 1   VIAFID ORCID Logo  ; Hadley, Caroline C 1 ; Bayley, James C, V 1 ; A Basit Khan 1 ; Jalali, Ali 1 ; Sweeney, Alex D 2 ; Klisch, Tiemo J 3   VIAFID ORCID Logo  ; Patel, Akash J 4   VIAFID ORCID Logo 

 Department of Neurosurgery, Baylor College of Medicine, Houston, TX 77030, USA; [email protected] (S.L.); [email protected] (P.J.K.); [email protected] (C.C.H.); [email protected] (J.C.B.V.); [email protected] (A.B.K.); [email protected] (A.J.) 
 Department of Otolaryngology-Head and Neck Surgery, Baylor College of Medicine, Houston, TX 77030, USA; [email protected] 
 Jan and Dan Duncan Neurological Research Institute, Texas Children’s Hospital, Houston, TX 77030, USA; [email protected]; Department of Molecular and Human Genetics, Baylor College of Medicine, Houston, TX 77030, USA 
 Department of Neurosurgery, Baylor College of Medicine, Houston, TX 77030, USA; [email protected] (S.L.); [email protected] (P.J.K.); [email protected] (C.C.H.); [email protected] (J.C.B.V.); [email protected] (A.B.K.); [email protected] (A.J.); Department of Otolaryngology-Head and Neck Surgery, Baylor College of Medicine, Houston, TX 77030, USA; [email protected]; Jan and Dan Duncan Neurological Research Institute, Texas Children’s Hospital, Houston, TX 77030, USA; [email protected] 
First page
1633
Publication year
2019
Publication date
2019
Publisher
MDPI AG
e-ISSN
20726694
Source type
Scholarly Journal
Language of publication
English
ProQuest document ID
2547543021
Copyright
© 2019 by the authors. Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (http://creativecommons.org/licenses/by/4.0/). Notwithstanding the ProQuest Terms and Conditions, you may use this content in accordance with the terms of the License.