A 2ND EWINGS-SARCOMA TRANSLOCATION, T(2122), FUSES THE EWS GENE TO ANOTHER ETS-FAMILY TRANSCRIPTION FACTOR, ERG

The t(11;22)(q24;q12), present in 85% of Ewing's sarcoma and related t umours, fuses the EWS gene from chromosome 22q12 and the ETS family me mber, FLI-1 This results in the expression of a chimaeric protein cont aining the amino-terminal portion of EWS fused to the ETS DNA-binding domain of FLI-1. We have identified a second Ewing's sarcoma transloca tion, t(21;22)(q22;q12), that fuses EWS to a different ETS family memb er, the ERG gene located on band 21q22. Identical EWS nucleotide seque nces found in the EWS/FLI-1 fusion transcripts are fused to portions o f ERG encoding an ETS DNA-binding domain resulting in expression of a hybrid EWS/ERG protein. These findings suggest that fusion of EWS to d ifferent members of the ETS family of transcription factor genes may r esult in the expression of similar disease phenotypes.