Abstract
The translocation t(12;16)(q13;p11), which cytogenetically characterizes myxoid liposarcomas (MLS), results in a fusion of the CHOP gene in 12q13 and the FUS gene in 16p11, creating a chimeric FUS/CHOP gene. We have identified two cases of MLS with translocations giving rise to recombination between 12q13 and 22q12. The result was a fusion of the N-terminal part of the EWS gene in 22q12, involved in a number of mesenchymal tumor types, with the CHOP gene and the creation of an EWS/CHOP chimeric gene. The presence of the EWS/CHOP chimeric gene in MLS shows that (i) the N-terminal part of FUS may be replaced by the N-terminal part of EWS in a CHOP fusion oncoprotein (ii) the two N-terminal parts, when fused to certain transcription factors, have a common or very similar oncogenic potential and (iii) the tumorigenic process in MLS and the morphogenetically distinctly different EWS-associated tumor types may be related.
Publication types
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Research Support, Non-U.S. Gov't
MeSH terms
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Amino Acid Sequence
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Base Sequence
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CCAAT-Enhancer-Binding Proteins*
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Cell Line
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Chimera
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Chromosome Mapping
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Chromosomes, Human, Pair 12*
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Chromosomes, Human, Pair 16*
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DNA Primers
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DNA-Binding Proteins / biosynthesis
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DNA-Binding Proteins / genetics*
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Heterogeneous-Nuclear Ribonucleoproteins
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Humans
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Karyotyping
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Leukemia, Myeloid, Acute / genetics
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Liposarcoma, Myxoid / genetics*
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Male
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Middle Aged
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Molecular Sequence Data
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Neoplasm Proteins / biosynthesis
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Neoplasm Proteins / genetics
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Nuclear Proteins / biosynthesis
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Nuclear Proteins / genetics
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Polymerase Chain Reaction
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RNA-Binding Protein EWS
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RNA-Binding Protein FUS
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Restriction Mapping
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Ribonucleoproteins / biosynthesis
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Ribonucleoproteins / genetics*
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Sarcoma / genetics
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Transcription Factor CHOP
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Transcription Factors / biosynthesis
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Transcription Factors / genetics*
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Transcription Factors / metabolism
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Translocation, Genetic*
Substances
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CCAAT-Enhancer-Binding Proteins
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DDIT3 protein, human
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DNA Primers
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DNA-Binding Proteins
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Heterogeneous-Nuclear Ribonucleoproteins
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Neoplasm Proteins
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Nuclear Proteins
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RNA-Binding Protein EWS
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RNA-Binding Protein FUS
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Ribonucleoproteins
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Transcription Factors
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Transcription Factor CHOP