Abstract
Background
Neurofibromatosis type 1 (NF1) is an autosomal dominant genetic disorder that affects nearly 1 in 3000 infants. Neurofibromin inactivation and NF1 gene mutations are involved in various aspects of neuronal function regulation, including neuronal development induction, electrophysiological activity elevation, growth factor expression, and neurotransmitter release. NF1 patients often exhibit a predisposition to tumor development, especially in the nervous system, resulting in the frequent occurrence of peripheral nerve sheath tumors and gliomas. Recent evidence suggests that nerves play a role in the development of multiple tumor types, prompting researchers to investigate the nerve as a vital component in and regulator of the initiation and progression of NF1-related nervous system tumors.
Conclusion
In this review, we summarize existing evidence about the specific effects of NF1 mutation on neurons and emerging research on the role of nerves in neurological tumor development, promising a new set of selective and targeted therapies for NF1-related tumors.
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References
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This work was supported by grants from National Natural Science Foundation of China (82102344; 82172228); Shanghai Rising Star Program supported by Science and Technology Commission of Shanghai Municipality (20QA1405600); Science and Technology Commission of Shanghai Mu-nicipality (19JC1413); Natural Science Foundation of Shanghai (22ZR1422300); “Chenguang Pro-gram” supported by Shanghai Education Development Foundation (SHEDF) (19CG18); Shanghai Municipal Key Clinical Specialty (shslczdzk00901); Innovative research team of high-level local universities in Shanghai (SSMU-ZDCX20180700).
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Conceptualization, ZC W, QF L, and HB Z; methodology, ZC W, QF L, and HB Z; investigation, LL G and MY X; resources, LL G and MY X; data curation, LL G and MY X; writing—original draft preparation, LL G; writing—review and editing, MY X, ZC W, and HB Z; visualization, LL G; supervision, ZC W, QF L, and HB Z; project administration, ZC W, QF L, and HB Z; funding acquisition, ZC W. All authors have read and agreed to the published version of the manuscript.
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Ge, LL., Xing, MY., Zhang, HB. et al. Role of nerves in neurofibromatosis type 1-related nervous system tumors. Cell Oncol. 45, 1137–1153 (2022). https://doi.org/10.1007/s13402-022-00723-3
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DOI: https://doi.org/10.1007/s13402-022-00723-3