Fusion of the EWS and CHOP genes in myxoid liposarcoma.
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.
Panagopoulos
I
I
Department of Clinical Genetics, Lund University Hospital, Sweden.
Höglund
M
M
Mertens
F
F
Mandahl
N
N
Mitelman
F
F
Aman
P
P
D000595:Amino Acid Sequence; D001483:Base Sequence; D022762:CCAAT-Enhancer-Binding Proteins; D002460:Cell Line; D002678:Chimera; D002874:Chromosome Mapping; D002881:Chromosomes, Human, Pair 12; D002885:Chromosomes, Human, Pair 16; D017931:DNA Primers; D004268:DNA-Binding Proteins / Q000096:biosynthesis / Q000235:genetics*; D034441:Heterogeneous-Nuclear Ribonucleoproteins; D006801:Humans; D007621:Karyotyping; D015470:Leukemia, Myeloid, Acute / Q000235:genetics; D018208:Liposarcoma, Myxoid / Q000235:genetics*; D008297:Male; D008875:Middle Aged; D008969:Molecular Sequence Data; D009363:Neoplasm Proteins / Q000096:biosynthesis / Q000235:genetics; D009687:Nuclear Proteins / Q000096:biosynthesis / Q000235:genetics; D016133:Polymerase Chain Reaction; D034802:RNA-Binding Protein EWS; D034702:RNA-Binding Protein FUS; D015183:Restriction Mapping; D012261:Ribonucleoproteins / Q000096:biosynthesis / Q000235:genetics*; D012509:Sarcoma / Q000235:genetics; D051742:Transcription Factor CHOP; D014157:Transcription Factors / Q000096:biosynthesis / Q000235:genetics* / Q000378:metabolism; D014178:Translocation, Genetic
D022762:CCAAT-Enhancer-Binding Proteins; C497249:DDIT3 protein, human; D017931:DNA Primers; D004268:DNA-Binding Proteins; D034441:Heterogeneous-Nuclear Ribonucleoproteins; D009363:Neoplasm Proteins; D009687:Nuclear Proteins; D034802:RNA-Binding Protein EWS; D034702:RNA-Binding Protein FUS; D012261:Ribonucleoproteins; D014157:Transcription Factors; D051742:Transcription Factor CHOP