The MYCN Protein in Health and Disease.
The MYCN Protein in Health and Disease.
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DOI：
10.3390/genes8040113
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发表时间：
2017-03-30
期刊：
影响因子：
3.5
通讯作者：
Arsenian-Henriksson M
中科院分区：
文献类型：
--
作者：
Ruiz-Pérez MV;Henley AB;Arsenian-Henriksson M
MYCN是原癌基因MYC家族的成员。它编码一种转录因子MYCN，参与胚胎发育过程中基本过程的控制。MYCN蛋白位于促进不同发育器官和组织中祖细胞的细胞生长、增殖和代谢的几种信号传导途径的下游。相反，MYCN信号失调支持几种不同肿瘤的发展，主要是儿童期发病，包括神经母细胞瘤，成神经管细胞瘤，横纹肌肉瘤和Wilms肿瘤，但它也与成年期发生的一些癌症有关，如前列腺癌和肺癌。在神经母细胞瘤中，MYCN扩增是与不良预后和治疗失败相关的最一致的遗传畸变。靶向MYCN已被提出作为治疗这些肿瘤的治疗策略，并且巨大的努力已经允许开发具有潜在临床用途的直接和间接MYCN抑制剂。
MYCN is a member of the MYC family of proto-oncogenes. It encodes a transcription factor, MYCN, involved in the control of fundamental processes during embryonal development. The MYCN protein is situated downstream of several signaling pathways promoting cell growth, proliferation and metabolism of progenitor cells in different developing organs and tissues. Conversely, deregulated MYCN signaling supports the development of several different tumors, mainly with a childhood onset, including neuroblastoma, medulloblastoma, rhabdomyosarcoma and Wilms’ tumor, but it is also associated with some cancers occurring during adulthood such as prostate and lung cancer. In neuroblastoma, MYCN-amplification is the most consistent genetic aberration associated with poor prognosis and treatment failure. Targeting MYCN has been proposed as a therapeutic strategy for the treatment of these tumors and great efforts have allowed the development of direct and indirect MYCN inhibitors with potential clinical use.