Shrestha Sumana, Morcavallo Alaide, Gorrini Chiara, Chesler Louis
Division of Clinical Studies, Institute of Cancer Research (ICR), London and Royal Marsden NHS Trust, Sutton, United Kingdom.
Division of Cancer Therapeutics, The Institute of Cancer Research (ICR), and The Royal Marsden NHS Trust, Sutton, United Kingdom.
Front Oncol. 2021 Jun 14;11:694320. doi: 10.3389/fonc.2021.694320. eCollection 2021.
The constitutive and dysregulated expression of the transcription factor MYCN has a central role in the pathogenesis of the paediatric brain tumour medulloblastoma, with an increased expression of this oncogene correlating with a worse prognosis. Consequently, the genomic and functional alterations of MYCN represent a major therapeutic target to attenuate tumour growth in medulloblastoma. This review will provide a comprehensive synopsis of the biological role of MYCN and its family components, their interaction with distinct signalling pathways, and the implications of this network in medulloblastoma development. We will then summarise the current toolbox for targeting MYCN and highlight novel therapeutic avenues that have the potential to results in better-tailored clinical treatments.
转录因子MYCN的组成性和失调表达在儿童脑肿瘤髓母细胞瘤的发病机制中起着核心作用，该致癌基因的表达增加与预后较差相关。因此，MYCN的基因组和功能改变是减轻髓母细胞瘤肿瘤生长的主要治疗靶点。本综述将全面概述MYCN及其家族成员的生物学作用、它们与不同信号通路的相互作用，以及该网络在髓母细胞瘤发展中的意义。然后，我们将总结目前针对MYCN的工具，并强调有可能带来更精准临床治疗的新治疗途径。
