A second Ewing's sarcoma translocation, t(21;22), fuses the EWS gene to another ETS-family transcription factor, ERG

Nat Genet. 1994 Feb;6(2):146-51. doi: 10.1038/ng0294-146.

Abstract

The t(11;22)(q24;q12), present in 85% of Ewing's sarcoma and related tumours, fuses the EWS gene from chromosome 22q12 and the ETS family member, FLI-1. This results in the expression of a chimaeric protein containing the amino-terminal portion of EWS fused to the ETS DNA-binding domain of FLI-1. We have identified a second Ewing's sarcoma translocation, t(21;22)(q22;q12), that fuses EWS to a different ETS family member, the ERG gene located on band 21q22. Identical EWS nucleotide sequences found in the EWS/FLI-1 fusion transcripts are fused to portions of ERG encoding an ETS DNA-binding domain resulting in expression of a hybrid EWS/ERG protein. These findings suggest that fusion of EWS to different members of the ETS family of transcription factor genes may result in the expression of similar disease phenotypes.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Amino Acid Sequence
  • Base Sequence
  • Child
  • Child, Preschool
  • Chromosomes, Human, Pair 21*
  • Chromosomes, Human, Pair 22*
  • Cloning, Molecular
  • DNA-Binding Proteins / analysis
  • DNA-Binding Proteins / genetics
  • Early Growth Response Protein 1
  • Female
  • Humans
  • Immediate-Early Proteins*
  • Karyotyping
  • Molecular Sequence Data
  • Precipitin Tests
  • Proto-Oncogene Protein c-fli-1
  • Proto-Oncogene Proteins*
  • RNA, Messenger / analysis
  • Recombinant Fusion Proteins / analysis
  • Recombinant Fusion Proteins / genetics
  • Retroviridae Proteins, Oncogenic / analysis
  • Retroviridae Proteins, Oncogenic / genetics
  • Sarcoma, Ewing / genetics*
  • Sequence Analysis, DNA
  • Trans-Activators / analysis
  • Trans-Activators / genetics
  • Transcription Factors / genetics*
  • Transcription, Genetic
  • Translocation, Genetic / genetics*
  • Tumor Cells, Cultured

Substances

  • DNA-Binding Proteins
  • EGR1 protein, human
  • Early Growth Response Protein 1
  • Immediate-Early Proteins
  • Proto-Oncogene Protein c-fli-1
  • Proto-Oncogene Proteins
  • RNA, Messenger
  • Recombinant Fusion Proteins
  • Retroviridae Proteins, Oncogenic
  • Trans-Activators
  • Transcription Factors

Associated data

  • GENBANK/S70579
  • GENBANK/S70593