Fusion of an ETS-family gene, EIAF, to EWS by t(17;22)(q12;q12) chromosome translocation in an undifferentiated sarcoma of infancy

✍ Scribed by Yasuhiko Kaneko; Koichi Yoshida; Masafumi Handa; Yasunori Toyoda; Hirokazu Nishihira; Yukichi Tanaka; Yoshiroh Sasaki; Setsuko Ishida; Fumihiro Higashino; Kei Fujinaga

• Publisher: John Wiley and Sons
• Year: 1996
• Tongue: English
• Weight: 489 KB
• Volume: 15
• Category: Article
• ISSN: 1045-2257
• DOI: 10.1002/(sici)1098-2264(199602)15:2<115::aid-gcc6>3.0.co;2-6

✦ Synopsis

E IAF is a newly isolated ETS-family gene that is located on I7q2 I and codes for the adenovirus E I A enhancer-binding protein.

In our chromosome analysis of I8 of the Ewing family of tumors and undifferentiated sarcomas, we found t( I7;22)(q I2;q 12) in an MlC2 antigen-positive undifferentiated sarcoma of infancy. On Southern blot analysis, EWS and ElAF 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. E IAF 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 We assume that the RNA binding domain of EWS may have been replaced by the DNA binding domain of E /AF in the EWS-E I AF fusion protein as in other fusion proteins previously characterized in Ewing sarcoma and other types of sarcomas.