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Review
. 2015 Jul 30;6(21):18265-75.
doi: 10.18632/oncotarget.4251.

Long noncoding RNAs and neuroblastoma

Gaurav Kumar Pandey  1 , Chandrasekhar Kanduri  1
Affiliations
Review

Long noncoding RNAs and neuroblastoma

Gaurav Kumar Pandey et al. Oncotarget. .

Abstract

Neuroblastoma is a disease that affects infants and despite intense multimodal therapy, high-risk patients have low survival rates (<50%). In recent years long noncoding RNAs (lncRNAs) have become the cutting edge of cancer research with inroads made in understanding their roles in multiple cancer types, including prostate and breast cancers. The roles of lncRNAs in neuroblastoma have just begun to be elucidated. This review summarises where we are with regards to lncRNAs in neuroblastoma. The known mechanistic roles of lncRNAs during neuroblastoma pathogenesis are discussed, as well as the relationship between lncRNA expression and the differentiation capacity of neuroblastoma cells. We speculate about the use of some of these lncRNAs, such as those mapping to the 6p22 hotspot, as biomarkers for neuroblastoma prognosis and treatment. This novel way of thinking about both neuroblastoma and lncRNAs brings a new perspective to the prognosis and treatment of high-risk patients.

Keywords: MYCN; NBAT1; neuroblastoma; neuronal differentiation; noncoding RNA.

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Figures

Figure 1
Figure 1. The 2p24 chromosomal region is amplified in several high-risk neuroblastoma patients and is associated with adverse outcomes
The amplified region harbors the MYCN oncogene and a lncRNA lncUSMycn, located 14 kb upstream of the MYCN oncogene. The lncUSMycn noncoding RNA post-transcriptionally regulates the expression of MYCN by acting as a scaffold for the RNA binding protein NonO and facilitating the interaction between NonO and MYCN. This RNA-protein interaction leads to the stabilization of the MYCN transcript and elevating the MYCN-driven transcriptional program in affected individuals.
Figure 2
Figure 2
A. In low-risk tumors, NBAT1 is expressed at higher levels due to a three dimensional chromosomal interaction between its hypomethylated promoter, the genotype (A/A; A/G) at SNP: 6939340 and unknown transcription factors. On the contrary, this interaction is disturbed in high-risk patients due to hypermethylation of the NBAT1 promoter and the presence of high-risk associated genotype (G/G) at the SNP: 6939340. B. NBAT1 is a tumor suppressor lncRNA and by interacting with chromatin remodeling protein EZH2, it controls tumor progression through suppressing oncogenic networks that drive proliferation and invasion of neuroblastoma cells. In addition, it drives neuroblastoma cells towards neural differentiation by suppressing the expression of NRSF/REST, which represses neural differentiation program in non-neuronal cells.
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