title: A variant Ewing's sarcoma translocation (7;22) fuses the EWS gene to the ETS gene ETV1.
abstract: Most Ewing's sarcomas or related primitive neuroectodermal tumors have the (11;22)(q24;q12) or less frequently the (21;22)(q22;q12) translocation. These rearrangements fuse the EWS gene on chromosome 22q12 to either the FLI1 or ERG genes, both members of the ETS family of transcription factors. Simple variant chromosomal translocations have been occasionally described in these tumors. We have identified a third Ewing's sarcoma translocation, the t(7;22)(p22;q12), that fuses EWS to the human homologue of the murine ETS gene ER81. This gene, designated ETV1 (for ETS Translocation Variant), is located on chromosome band 7p22. Identical EWS nucleotide sequences found in the majority of EWS-FLI1 and EWS-ERG chimeric transcripts are fused to a portion of ETV1 encoding an ETS domain with sequence specific DNA-binding activity. These findings confirm that the fusion of EWS to different ETS family members can result in a similar tumor phenotype.
authors.affiliation: Department of Experimental Oncology, St. Jude Children's Research Hospital, Memphis, Tennessee 38101.
authors.forename: I S
authors.initials: IS
authors.lastname: Jeon
authors.forename: J N
authors.initials: JN
authors.lastname: Davis
authors.forename: B S
authors.initials: BS
authors.lastname: Braun
authors.forename: J E
authors.initials: JE
authors.lastname: Sublett
authors.forename: M F
authors.initials: MF
authors.lastname: Roussel
authors.forename: C T
authors.initials: CT
authors.lastname: Denny
authors.forename: D N
authors.initials: DN
authors.lastname: Shapiro
mesh_terms: D000328:Adult; D000595:Amino Acid Sequence; D000818:Animals; D001483:Base Sequence; D002675:Child, Preschool; D002892:Chromosomes, Human, Pair 22; D002897:Chromosomes, Human, Pair 7; D003001:Cloning, Molecular; D018076:DNA, Complementary; D004268:DNA-Binding Proteins / Q000378:metabolism; D005260:Female; D005333:Fetus / Q000378:metabolism; D034441:Heterogeneous-Nuclear Ribonucleoproteins; D006801:Humans; D051379:Mice; D008969:Molecular Sequence Data; D009687:Nuclear Proteins / Q000235:genetics*; D012333:RNA, Messenger / Q000235:genetics / Q000378:metabolism; D034802:RNA-Binding Protein EWS; D016601:RNA-Binding Proteins / Q000235:genetics*; D011993:Recombinant Fusion Proteins / Q000235:genetics; D012261:Ribonucleoproteins / Q000235:genetics*; D012512:Sarcoma, Ewing / Q000235:genetics*; D017386:Sequence Homology, Amino Acid; D014157:Transcription Factors / Q000235:genetics*; D014178:Translocation, Genetic
chemical_list: D018076:DNA, Complementary; D004268:DNA-Binding Proteins; D034441:Heterogeneous-Nuclear Ribonucleoproteins; D009687:Nuclear Proteins; D012333:RNA, Messenger; D034802:RNA-Binding Protein EWS; D016601:RNA-Binding Proteins; D011993:Recombinant Fusion Proteins; D012261:Ribonucleoproteins; D014157:Transcription Factors