Glycine decarboxylase is a transcriptional target of MYCN required for neuroblastoma cell proliferation and tumorigenicity

Oncogene. 2019 Dec;38(50):7504-7520. doi: 10.1038/s41388-019-0967-3. Epub 2019 Aug 23.

Abstract

Genomic amplification of the oncogene MYCN is a major driver in the development of high-risk neuroblastoma, a pediatric cancer with poor prognosis. Given the challenge in targeting MYCN directly for therapy, we sought to identify MYCN-dependent metabolic vulnerabilities that can be targeted therapeutically. Here, we report that the gene encoding glycine decarboxylase (GLDC), which catalyzes the first and rate-limiting step in glycine breakdown with the production of the one-carbon unit 5,10-methylene-tetrahydrofolate, is a direct transcriptional target of MYCN. As a result, GLDC expression is markedly elevated in MYCN-amplified neuroblastoma tumors and cell lines. This transcriptional upregulation of GLDC expression is of functional significance, as GLDC depletion by RNA interference inhibits the proliferation and tumorigenicity of MYCN-amplified neuroblastoma cell lines by inducing G1 arrest. Metabolomic profiling reveals that GLDC knockdown disrupts purine and central carbon metabolism and reduces citrate production, leading to a decrease in the steady-state levels of cholesterol and fatty acids. Moreover, blocking purine or cholesterol synthesis recapitulates the growth-inhibitory effect of GLDC knockdown. These findings reveal a critical role of GLDC in sustaining the proliferation of neuroblastoma cells with high-level GLDC expression and suggest that MYCN amplification is a biomarker for GLDC-based therapeutic strategies against high-risk neuroblastoma.

Publication types

  • Research Support, N.I.H., Extramural

MeSH terms

  • Animals
  • Apoptosis / genetics
  • Biomarkers, Tumor / genetics*
  • Carcinogenesis / genetics
  • Cell Cycle Checkpoints / genetics
  • Cell Line, Tumor
  • Cell Proliferation / genetics
  • Gene Expression Regulation, Neoplastic / genetics
  • Glycine / metabolism
  • Glycine Dehydrogenase (Decarboxylating) / genetics*
  • Heterografts
  • Humans
  • Metabolomics
  • Mice
  • N-Myc Proto-Oncogene Protein / genetics*
  • Neuroblastoma / genetics*
  • Neuroblastoma / pathology
  • Purines / metabolism
  • Tetrahydrofolates / metabolism

Substances

  • Biomarkers, Tumor
  • MYCN protein, human
  • N-Myc Proto-Oncogene Protein
  • Purines
  • Tetrahydrofolates
  • 5,10-methenyltetrahydrofolate
  • Glycine Dehydrogenase (Decarboxylating)
  • Glycine
  • purine