Biology of EWS/ETS fusions in Ewing's family tumors

Oncogene. 2001 Sep 10;20(40):5747-54. doi: 10.1038/sj.onc.1204598.


Tumor-associated chromosomal translocations lead to the formation of chimeric fusions between the EWS gene and one of five different ETS transcription factors in Ewing's family tumors (EFTs). The resultant EWS/ETS proteins promote oncogenesis in a dominant fashion in model systems and are necessary for continued growth of EFT cell lines. EWS belongs to a family of genes that encode proteins that may serve as adapters between the RNA polymerase II complex and RNA splicing factors. EWS/ETS fusions have biochemical characteristics of aberrant transcription factors and appear to promote abnormal cellular growth by transcriptionally modulating a network of target genes. Early evidence suggests that EWS/ETS proteins may also impact gene expression through alteration in RNA processing. Elucidation of EWS/ETS target gene networks in the context of other signaling pathways will hopefully lead to biology based therapeutic strategies for EFT.

Publication types

  • Research Support, U.S. Gov't, P.H.S.
  • Review

MeSH terms

  • Cell Division
  • Heterogeneous-Nuclear Ribonucleoproteins
  • Karyotyping
  • Models, Biological
  • Models, Genetic
  • Multigene Family
  • Neoplasms / etiology
  • Neoplasms / genetics
  • Oncogene Proteins, Fusion / genetics*
  • Oncogene Proteins, Fusion / metabolism*
  • Protein Binding
  • Protein Structure, Tertiary
  • RNA / metabolism
  • RNA-Binding Protein EWS
  • Ribonucleoproteins / chemistry*
  • Ribonucleoproteins / genetics
  • Ribonucleoproteins / metabolism
  • Sarcoma, Ewing / genetics*
  • Translocation, Genetic


  • Heterogeneous-Nuclear Ribonucleoproteins
  • Oncogene Proteins, Fusion
  • RNA-Binding Protein EWS
  • Ribonucleoproteins
  • RNA