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神经母细胞瘤是儿童外周神经系统的肿瘤。在近20%的神经母细胞瘤病例中发现有原癌基因MYCN的扩增和过表达现象。近几年发现MYCN基因编码的N-myc 蛋白，不仅能够调控多种非编码RNA 的表达，而且也受非编码RNA的调控。miRNA 是在神经母细胞瘤中研究最广泛的非编码RNA，近年来研究发现N-myc 蛋白对于绝大部分miRNA 起抑制作用，如mir-184、 mir-542-5p 等，仅对少数miRNA起诱导表达的作用，如mir-17-92 基因簇、mir-9、mir-421 等。N-myc 蛋白不仅对miRNA 的表达起调节作用，而且也调控其它lncRNA 的表达，如T-UCR、NDM29、linc00467、MALAT1。有趣的是，N-myc 蛋白和miRNA 之间的作用是相互的，它们可以双向调节，其调控网络也是复杂的。miRNA 能够靶向MYCN，同时MYCN也能够调控miRNA。该文对神经母细胞瘤中N-myc蛋白和非编码RNA 间的相互影响，以及两者之间的关系对神经母细胞瘤的发生有何作用作一综述。

英文摘要:

Neuroblastoma is a pediatric tumor of the sympathetic nervous system. Amplification and overexpression of the MYCN proto-oncogene occurs in approximately 20% of neuroblastomas. MYCN encodes the transcriptional regulator N-myc. In recent years, it has become clear that N-myc not only modulates the expression of several classes of noncoding RNAs, but noncoding RNAs have also been documented to modulates N-myc expression. MicroRNAs are the most widely studied noncoding RNA molecules in neuroblastoma. Growing evidences show that N-myc predominantly acts repressively on the most miRNA composition (i.e., mir-184, mir-542-5p, etc.), only induces less miRNA expression (i.e., mir-17-92 cluster, mir-9, mir-421, etc.). The N-myc protein is not only considered as an important regulator of several miRNAs, but also regulates the expression of long noncoding RNAs such as T-UCR、 NDM29、linc00467 and MALAT1. The interaction between N-myc and miRNAs is mutual and complex, they can regulate to each other. MYCN can regulate the expression of miRNAs, but itself is targeted by miRNAs. This review summarizes the current knowledge about the interplay between N-myc and noncoding RNAs in neuroblastoma and how this contributes to neuroblastoma tumorigenesis.

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