title: A second Ewing's sarcoma translocation, t(21;22), fuses the EWS gene to another ETS-family transcription factor, ERG.
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.
authors.affiliation: Department of Pathology and Laboratory Medicine, Childrens Hospital of Los Angeles/University of Southern California 90027.
authors.forename: P H
authors.initials: PH
authors.lastname: Sorensen
authors.forename: S L
authors.initials: SL
authors.lastname: Lessnick
authors.forename: D
authors.initials: D
authors.lastname: Lopez-Terrada
authors.forename: X F
authors.initials: XF
authors.lastname: Liu
authors.forename: T J
authors.initials: TJ
authors.lastname: Triche
authors.forename: C T
authors.initials: CT
authors.lastname: Denny
mesh_terms: D000595:Amino Acid Sequence; D001483:Base Sequence; D002648:Child; D002675:Child, Preschool; D002891:Chromosomes, Human, Pair 21; D002892:Chromosomes, Human, Pair 22; D003001:Cloning, Molecular; D004268:DNA-Binding Proteins / Q000032:analysis / Q000235:genetics; D051766:Early Growth Response Protein 1; D005260:Female; D006801:Humans; D017874:Immediate-Early Proteins; D007621:Karyotyping; D008969:Molecular Sequence Data; D011233:Precipitin Tests; D051789:Proto-Oncogene Protein c-fli-1; D011518:Proto-Oncogene Proteins; D012333:RNA, Messenger / Q000032:analysis; D011993:Recombinant Fusion Proteins / Q000032:analysis / Q000235:genetics; D015682:Retroviridae Proteins, Oncogenic / Q000032:analysis / Q000235:genetics; D012512:Sarcoma, Ewing / Q000235:genetics*; D017422:Sequence Analysis, DNA; D015534:Trans-Activators / Q000032:analysis / Q000235:genetics; D014157:Transcription Factors / Q000235:genetics*; D014158:Transcription, Genetic; D014178:Translocation, Genetic / Q000235:genetics*; D014407:Tumor Cells, Cultured
chemical_list: D004268:DNA-Binding Proteins; C497325:EGR1 protein, human; D051766:Early Growth Response Protein 1; D017874:Immediate-Early Proteins; D051789:Proto-Oncogene Protein c-fli-1; D011518:Proto-Oncogene Proteins; D012333:RNA, Messenger; D011993:Recombinant Fusion Proteins; D015682:Retroviridae Proteins, Oncogenic; D015534:Trans-Activators; D014157:Transcription Factors