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Updated: Jan 26, 2026

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Foxr2 promotes formation of CNS-embryonal tumors in a Trp53-deficient background

Boonmin Poh1, Hideto Koso1, Hiroyuki Momota2

  • 1Division of Molecular and Developmental Biology, The Institute of Medical Science, The University of Tokyo, Tokyo, Japan.

Neuro-Oncology
|April 13, 2019
PubMed
Abstract

Insights

Forkhead box R2 (Foxr2) drives the formation of aggressive pediatric brain tumors, specifically central nervous system (CNS) embryonal tumors. This oncogene promotes tumor development and expansion in mouse models, highlighting its critical role.

Area of Science:

  • Neuro-oncology
  • Pediatric oncology
  • Molecular oncology

Background:

  • Embryonal tumors in the central nervous system (CNS) are aggressive pediatric brain tumors.
  • Forkhead box R2 (Foxr2) was identified as an oncogene in medulloblastoma.
  • Foxr2 translocation is observed in CNS neuroblastoma with Foxr2 activation (CNS NB-Foxr2), but its in vivo function is unknown.

Purpose of the Study:

  • To investigate the in vivo function of Foxr2 in CNS tumor formation.
  • To analyze the oncogenic role of Foxr2 in a Trp53-deficient mouse model.

Main Methods:

  • Generated transgenic mice overexpressing Foxr2 in a Trp53-deficient background.
  • Performed histological analysis and characterized tumor-derived sphere-forming cells.
  • Investigated gene expression profiles of tumor-derived cells.

Main Results:

  • Foxr2 overexpression and Trp53 loss promoted CNS embryonal tumor formation in the olfactory bulb and brainstem.
  • Tumors exhibited features of small round blue cell tumors with neuronal and glial differentiation.
  • Tumor-derived cells possessed tumor-initiating properties and gene expression profiles consistent with CNS NB-Foxr2.

Conclusions:

  • Foxr2 plays a causative role in the development of CNS embryonal tumors.
  • Foxr2 promotes the expansion of oligodendrocyte precursor cells in the olfactory bulb.
Keywords:
CNS NB-Foxr2CNS-embryonal tumorFoxr2PNETmedulloblastoma

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