Cell-cycle dependent expression of a translocation-mediated fusion oncogene mediates checkpoint adaptation in rhabdomyosarcoma

PLoS Genet. 2014 Jan;10(1):e1004107. doi: 10.1371/journal.pgen.1004107. Epub 2014 Jan 16.

Abstract

Rhabdomyosarcoma is the most commonly occurring soft-tissue sarcoma in childhood. Most rhabdomyosarcoma falls into one of two biologically distinct subgroups represented by alveolar or embryonal histology. The alveolar subtype harbors a translocation-mediated PAX3:FOXO1A fusion gene and has an extremely poor prognosis. However, tumor cells have heterogeneous expression for the fusion gene. Using a conditional genetic mouse model as well as human tumor cell lines, we show that that Pax3:Foxo1a expression is enriched in G2 and triggers a transcriptional program conducive to checkpoint adaptation under stress conditions such as irradiation in vitro and in vivo. Pax3:Foxo1a also tolerizes tumor cells to clinically-established chemotherapy agents and emerging molecularly-targeted agents. Thus, the surprisingly dynamic regulation of the Pax3:Foxo1a locus is a paradigm that has important implications for the way in which oncogenes are modeled in cancer cells.

Publication types

  • Research Support, N.I.H., Extramural
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adaptation, Physiological / radiation effects
  • Animals
  • Cell Cycle Checkpoints / genetics
  • Cell Cycle Checkpoints / radiation effects
  • Cell Line, Tumor
  • Forkhead Box Protein O1
  • Forkhead Transcription Factors / biosynthesis*
  • Forkhead Transcription Factors / genetics
  • Gene Expression Regulation, Neoplastic / radiation effects
  • Humans
  • Mice
  • Oncogene Proteins, Fusion / biosynthesis
  • Oncogene Proteins, Fusion / genetics*
  • PAX3 Transcription Factor
  • Paired Box Transcription Factors / biosynthesis*
  • Paired Box Transcription Factors / genetics
  • Radiation
  • Rhabdomyosarcoma / genetics*
  • Rhabdomyosarcoma / pathology
  • Translocation, Genetic

Substances

  • FOXO1 protein, human
  • Forkhead Box Protein O1
  • Forkhead Transcription Factors
  • Oncogene Proteins, Fusion
  • PAX3 Transcription Factor
  • PAX3 protein, human
  • Paired Box Transcription Factors

Associated data

  • GEO/GSE41675