title: Fusion of the EWS-related gene TAF2N to TEC in extraskeletal myxoid chondrosarcoma.
abstract: Extraskeletal myxoid chondrosarcomas (EMCs) are characterized by a recurrent t(9;22)(q22;q12) translocation, resulting in the fusion of the EWS gene in 22q12 and the TEC gene in 9q22. Here we report that a third member of the EWS, TLS/FUS gene family, TAF2N, can replace EWS as a fusion partner to TEC in EMC. Two tumors, one with a novel t(9;17)(q22;q11) variant translocation and one with an apparently normal karyotype, expressed TAF2N-TEC fusion transcripts. In both cases, the chimeric transcripts were shown to contain exon 6 of TAF2N fused to the entire coding region of TEC. This transcript is structurally and functionally very similar to the EWS-TEC fusions. The exchange of the EWS NH2-terminal part with the TAF2N NH2-terminal part in EMC further underscores the oncogenic potential of these protein domains as partners in fusion genes.
authors.affiliation: Department of Pathology, Göteborg University, Sweden.
authors.forename: H
authors.initials: H
authors.lastname: Sjögren
authors.forename: J
authors.initials: J
authors.lastname: Meis-Kindblom
authors.forename: L G
authors.initials: LG
authors.lastname: Kindblom
authors.forename: P
authors.initials: P
authors.lastname: Aman
authors.forename: G
authors.initials: G
authors.lastname: Stenman
mesh_terms: D000368:Aged; D000595:Amino Acid Sequence; D019677:Artificial Gene Fusion; D001483:Base Sequence; D002813:Chondrosarcoma / Q000235:genetics*; D002874:Chromosome Mapping; D004268:DNA-Binding Proteins / Q000235:genetics*; D034441:Heterogeneous-Nuclear Ribonucleoproteins; D006801:Humans; D008297:Male; D008875:Middle Aged; D008969:Molecular Sequence Data; D009419:Nerve Tissue Proteins; D009687:Nuclear Proteins / Q000235:genetics*; D012333:RNA, Messenger / Q000032:analysis; D034802:RNA-Binding Protein EWS; D034702:RNA-Binding Protein FUS; D011987:Receptors, Steroid; D011988:Receptors, Thyroid Hormone; D012261:Ribonucleoproteins / Q000235:genetics; D035381:TATA-Binding Protein Associated Factors; D014157:Transcription Factors / Q000235:genetics*
chemical_list: D004268:DNA-Binding Proteins; D034441:Heterogeneous-Nuclear Ribonucleoproteins; C490412:NR4A3 protein, human; D009419:Nerve Tissue Proteins; D009687:Nuclear Proteins; D012333:RNA, Messenger; D034802:RNA-Binding Protein EWS; D034702:RNA-Binding Protein FUS; D011987:Receptors, Steroid; D011988:Receptors, Thyroid Hormone; D012261:Ribonucleoproteins; C102041:TAF15 protein, human; D035381:TATA-Binding Protein Associated Factors; D014157:Transcription Factors