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. 2023 Aug 16;5(1):vdad099. doi: 10.1093/noajnl/vdad099

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© The Author(s) 2023. Published by Oxford University Press, the Society for Neuro-Oncology and the European Association of Neuro-Oncology.

This is an Open Access article distributed under the terms of the Creative Commons Attribution License (https://creativecommons.org/licenses/by/4.0/), which permits unrestricted reuse, distribution, and reproduction in any medium, provided the original work is properly cited.

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Figure 2. — Signaling pathways regulated by Merlin are shown along with therapeutic targets and their targeted agent. Merlin regulates the activity of the receptor tyrosine kinases and inhibits their downstream signaling pathways. Merlin inhibits the PI3K-AKT-mTORC1 pathway through PIKE-L and through mTORC1. Merlin inhibits the RAS-Raf-MEK1/2 pathway as well. In this manner, it regulates cell proliferation, cell growth, and survival. In the absence of merlin in vestibular schwannomas in patients with NF2-related Schwannomatosis there is increased activity in these pathways leading to tumor growth. In such a situation, these therapeutic agents can act on their targets to regulate these pathways and reduce cell growth and proliferation, leading to tumor control.