MYCN Function in Neuroblastoma Development.

MYCN Function in Neuroblastoma Development.
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DOI:
10.3389/fonc.2020.624079
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发表时间:
2020
影响因子:
4.7
通讯作者:
Johnsen JI
Johnsen JI
中科院分区:
医学3区
文献类型:
--
作者:
Otte J;Dyberg C;Pepich A;Johnsen JI

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转录因子MYCN的表达异常在神经系统肿瘤如儿童神经母细胞瘤中经常被检测到。在这里,MYCN基因扩增是在神经嵴源性细胞中诱导肿瘤转化的单一致癌驱动因素。这种异常的MYCN表达是不良预后的最强预测因子之一。它在诊断时就存在,而在MYCN非扩增型神经母细胞瘤的后期肿瘤发生过程中从未获得过。这表明MYCN表达的增加是这些癌症的早期事件,导致细胞的特殊失调,导致胚胎或癌症干细胞样特性,如自我更新增强、凋亡抵抗和代谢灵活性。
Dysregulated expression of the transcription factor MYCN is frequently detected in nervous system tumors such as childhood neuroblastoma. Here, gene amplification of MYCN is a single oncogenic driver inducing neoplastic transformation in neural crest-derived cells. This abnormal MYCN expression is one of the strongest predictors of poor prognosis. It is present at diagnosis and is never acquired during later tumorigenesis of MYCN non-amplified neuroblastoma. This suggests that increased MYCN expression is an early event in these cancers leading to a peculiar dysregulation of cells that results in embryonal or cancer stem-like qualities, such as increased self-renewal, apoptotic resistance, and metabolic flexibility.
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