EWS-FLI1 in Ewing's sarcoma: real targets and collateral damage

Adv Exp Med Biol. 2006:587:41-52. doi: 10.1007/978-1-4020-5133-3_4.

Abstract

Ewing's sarcoma family of tumors (ESFT) are a clinically and scientifically very demanding group of tumors in children and young adults with still unknown histogenesis. The rate-limiting oncogenic mutation in this disease has been identified as a chromosomal translocation, t(11;22)(q24;q12), that leads to the expression of a chimeric transcription factor, EWS-FLI1. We have studied the downstream pathway of EWS-FLI1 by a dual strategy including the isolation of direct target genes from ESFT chromatin and the monitoring of transcriptomic changes after silencing of EWS-FLI1 by RNA interference. This study has lead to the identification of several directly EWS-FLI1-regulated genes and the characterization of their genomic distribution. By comparing several ESFT cell lines, not only variation in overall gene expression patterns downstream of EWS-FLIl was observed, but also differential regulation of directly EWS-FLI1-bound genes. Interestingly, there was variation between members of the same functional gene families. Studies on CD99, another diagnostic hallmark of ESFT, in relation to EWS-FLI1 provided additional evidence for context dependence of fusion protein function. Together, our study represents a first approach to the separation of essential molecular consequences from noise generated by the EWS-FLI1 gene rearrangement in ESFT.

Publication types

  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Bone Neoplasms / genetics
  • Bone Neoplasms / physiopathology*
  • Gene Expression Regulation, Neoplastic
  • Humans
  • Oncogene Proteins, Fusion / genetics
  • Oncogene Proteins, Fusion / physiology*
  • Proto-Oncogene Protein c-fli-1 / genetics*
  • Proto-Oncogene Protein c-fli-1 / physiology*
  • RNA-Binding Protein EWS / genetics*
  • Sarcoma, Ewing / genetics
  • Sarcoma, Ewing / physiopathology*
  • Translocation, Genetic

Substances

  • EWS-FLI fusion protein
  • FLI1 protein, human
  • Oncogene Proteins, Fusion
  • Proto-Oncogene Protein c-fli-1
  • RNA-Binding Protein EWS