Identification of genes regulated by the EWS/NR4A3 fusion protein in extraskeletal myxoid chondrosarcoma

Tumour Biol. 2012 Oct;33(5):1599-605. doi: 10.1007/s13277-012-0415-2. Epub 2012 May 18.

Abstract

Approximately 75 % of extraskeletal myxoid chondrosarcoma tumors (EMC) harbor a t(9;22) chromosome translocation generating an EWS/NR4A3 fusion protein that is thought to be instrumental in the tumoral process. Current evidence suggests that one function of the fusion protein is to overexpress target genes. We have generated an in vitro human cellular model in which the fusion protein is expressed in mesenchymal bone marrow stem cells. We have performed microarray analyses of these cells and identified several genes overexpressed in the presence of EWS/NR4A3 which are also overexpressed in EMC tumors. These genes and their products represent potential therapeutic targets for EMC tumors.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Animals
  • COS Cells
  • Cell Line, Tumor
  • Chlorocebus aethiops
  • Chondrosarcoma / genetics*
  • DNA-Binding Proteins / genetics*
  • Gene Expression Profiling
  • Gene Expression Regulation, Neoplastic
  • Humans
  • Oncogene Proteins, Fusion / genetics*
  • RNA-Binding Protein EWS / genetics*
  • Receptors, Steroid / genetics*
  • Receptors, Thyroid Hormone / genetics*
  • Trans-Activators / genetics*

Substances

  • DNA-Binding Proteins
  • EWS-TEC fusion protein, human
  • NR4A3 protein, human
  • Oncogene Proteins, Fusion
  • RNA-Binding Protein EWS
  • Receptors, Steroid
  • Receptors, Thyroid Hormone
  • Trans-Activators