A novel zinc finger gene is fused to EWS in small round cell tumor

Tiziana Mastrangelo, Piergiorgio Modena, Silvana Tornielli, Florentia Bullrich, Maria Adele Testi, Alessandra Mezzelani, Paolo Radice, Alberto Azzarelli, Silvana Pilotti, Carlo M. Croce, Marco A. Pierotti, Gabriella Sozzi

Research output: Contribution to journalArticle

Abstract

Ewing sarcoma family of tumors share recurrent translocations that fuse EWS from 22q12 to five different members of transcription factors namely FLI1, ERG, ETV1, E1AF and FEV. Different classes of DNA binding proteins, ATF1, WT1 and CHOP are fused to EWS generating distinct tumor phenotypes: clear cell sarcoma, desmoplastic small round cell tumor, and myxoid liposarcoma, respectively. We have cloned a novel gene located at 22q12 fused to EWS by a submicroscopic inversion of 22q in a small round cell sarcoma showing a translocation (t(1;22)(p36.1;q12). The gene, designated ZSG (Zinc finger Sarcoma Gene), is a putative Cys2-His2 zinc finger protein which contains a POZ transcriptional repressor-like domain at the N-terminus. The rearrangement involves intron 8 of EWS and exon 1 of ZSG creating a chimeric sequence containing the transactivation domain of EWS fused to zinc finger domain of ZSG. This product lacks the transcriptional repressor domain at the N-terminus of ZSG. A rearrangement of the second ZSG allele was also found in tumor cells. This is the first example of an intra-chromosomal rearrangement of chromosome 22, undetectable by cytogenetics, activating EWS in soft tissue sarcoma.

Original languageEnglish
Pages (from-to)3799-3804
Number of pages6
JournalOncogene
Volume19
Issue number33
Publication statusPublished - Aug 3 2000

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Keywords

  • EWS
  • Fusion
  • POZ/BTB domain
  • Sarcoma
  • Zinc finger

ASJC Scopus subject areas

  • Molecular Biology
  • Cancer Research
  • Genetics

Cite this

Mastrangelo, T., Modena, P., Tornielli, S., Bullrich, F., Testi, M. A., Mezzelani, A., Radice, P., Azzarelli, A., Pilotti, S., Croce, C. M., Pierotti, M. A., & Sozzi, G. (2000). A novel zinc finger gene is fused to EWS in small round cell tumor. Oncogene, 19(33), 3799-3804.