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.
Publication types
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Research Support, Non-U.S. Gov't
MeSH terms
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Aged
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Amino Acid Sequence
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Artificial Gene Fusion
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Base Sequence
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Chondrosarcoma / genetics*
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Chromosome Mapping
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DNA-Binding Proteins / genetics*
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Heterogeneous-Nuclear Ribonucleoproteins
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Humans
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Male
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Middle Aged
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Molecular Sequence Data
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Nerve Tissue Proteins*
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Nuclear Proteins / genetics*
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RNA, Messenger / analysis
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RNA-Binding Protein EWS
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RNA-Binding Protein FUS
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Receptors, Steroid
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Receptors, Thyroid Hormone
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Ribonucleoproteins / genetics
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TATA-Binding Protein Associated Factors*
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Transcription Factors / genetics*
Substances
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DNA-Binding Proteins
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Heterogeneous-Nuclear Ribonucleoproteins
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NR4A3 protein, human
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Nerve Tissue Proteins
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Nuclear Proteins
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RNA, Messenger
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RNA-Binding Protein EWS
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RNA-Binding Protein FUS
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Receptors, Steroid
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Receptors, Thyroid Hormone
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Ribonucleoproteins
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TAF15 protein, human
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TATA-Binding Protein Associated Factors
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Transcription Factors