Abstract
EIAF is a newly isolated ETS-family gene that is located on 17q21 and codes for the adenovirus EIA enhancer-binding protein. In our chromosome analysis of 18 of the Ewing family of tumors and undifferentiated sarcomas, we found t(17;22)(q12;q12) in an MIC2 antigen-positive undifferentiated sarcoma of infancy. On Southern blot analysis, EWS and EIAF cDNA probes hybridized to the same rearranged band, indicating that an EWS-EIAF fusion gene was formed in the tumor. Further Southern blot analysis using four EIAF cDNA probes of different sizes showed that the breakpoint lies in the region upstream to the ETS domain of the EIAF gene. EIAF may be the fourth ETS-family gene to be identified forming a fusion gene with EWS. We assume that the RNA binding domain of EWS may have been replaced by the DNA binding domain of EIAF in the EWS-EIAF fusion protein as in other fusion proteins previously characterized in Ewing sarcoma and other types of sarcomas.
Publication types
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Case Reports
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Research Support, Non-U.S. Gov't
MeSH terms
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Adenovirus E1A Proteins / genetics*
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Cheek
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Chromosomes, Human, Pair 17 / genetics*
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Chromosomes, Human, Pair 17 / ultrastructure
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Chromosomes, Human, Pair 22 / genetics*
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Chromosomes, Human, Pair 22 / ultrastructure
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DNA, Neoplasm / genetics
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Facial Neoplasms / congenital
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Facial Neoplasms / genetics*
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Facial Neoplasms / pathology
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Fatal Outcome
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Female
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Heterogeneous-Nuclear Ribonucleoproteins
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Humans
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Infant, Newborn
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Multigene Family*
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Oncogene Proteins, Fusion / genetics*
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Proto-Oncogene Proteins / genetics*
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Proto-Oncogene Proteins c-ets
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RNA-Binding Protein EWS
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Ribonucleoproteins / genetics*
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Sarcoma / congenital
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Sarcoma / genetics*
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Sarcoma / pathology
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Translocation, Genetic / genetics*
Substances
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Adenovirus E1A Proteins
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DNA, Neoplasm
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ETV4 protein, human
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Heterogeneous-Nuclear Ribonucleoproteins
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Oncogene Proteins, Fusion
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Proto-Oncogene Proteins
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Proto-Oncogene Proteins c-ets
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RNA-Binding Protein EWS
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Ribonucleoproteins