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Neuroblastoma-derived v-myc avian myelocytomatosis viral related oncogene or MYCN gene
  1. Neha Bhardwaj1,
  2. Gargi Das2,
  3. Radhika Srinivasan3
  1. 1 Department of Pathology, Post Graduate Institute of Medical Education and Research, Chandigarh, India
  2. 2 Medical Oncology (Pediatric Oncology), Cancer Institute-WIA, Chennai, Tamil Nadu, India
  3. 3 Pathology, Post Graduate Institute of Medical Education and Research, Chandigarh, India
  1. Correspondence to Dr Radhika Srinivasan, Pathology, Post Graduate Institute of Medical Education and Research, Chandigarh 160012, India; drsradhika{at}gmail.com

Abstract

The MYCN gene belongs to the MYC family of transcription factors. Amplification of MYCN, first discovered in neuroblastoma cells, ushered in the era of cancer genomics. The MYCN gene and MYCN protein are extensively studied in the context of neuroblastoma. As demonstrated in transgenic mouse models, MYCN gene shows a restricted spatiotemporal expression predominantly in the neural crest cells which explains the associated neoplasms including neuroblastoma and central nervous system tumours. In neuroblastoma, MYCN amplification is a marker of aggressive tumours with poor prognosis and survival and forms the basis of risk stratification classifications.

MYCN dysregulated expression occurs by several mechanisms at the transcriptional, translational and post-translational levels. These include massive gene amplification which occurs in an extrachromosomal location, upregulated transcription and stabilisation of the protein increasing its half-life. MYCN protein, a basic loop-helix-loop leucine zipper transcription factor, has many regions which bind to several proteins foremost of which is MAX forming the MYC:MAX heterodimer. Overall, MYCN controls multiple aspects of cell fate, foremost of which is cellular proliferation besides cell differentiation, apoptosis and cellular metabolism, all of which are the focus of this brief review. In addition to amplification, other mechanisms of MYCN overexpression include activating missense mutations as reported in basal cell carcinoma and Wilms tumour. A better understanding of this molecule will help in the discovery of novel strategies for its indirect targeting to improve the outcomes of patients with neuroblastoma and other MYCN-associated neoplasms.

  • molecular biology
  • neoplasms
  • oncogenes
  • pathology, molecular

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Footnotes

  • Handling editor Vikram Deshpande.

  • Twitter @drsradhika

  • Contributors Review article conceptualised by NB, GD and RS. NB and GD performed the literature review and prepared the initial draft. Manuscript was edited by RS. Figures 1 and 2 were prepared by GD and figure 3 by NB and edited by RS.

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Competing interests None declared.

  • Provenance and peer review Commissioned; externally peer reviewed.