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

Oncogene volume 19, pages 3799–3804 (2000)Cite this article

Abstract

Ewing sarcoma family of tumors share recurrent translocations that fuse EWS from 22q12 to five different members of transcription factors namely FLI-1, 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.

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Acknowledgements

We are grateful to Mr Mario Azzini for artwork preparation and to Mrs Silvia Grassi for the editing of the manuscript. This work is supported partially by the Associazione and Fondazione Italiana per la Ricerca sul Cancro (AIRC/FIRC).

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Author notes

  1. Tiziana Mastrangelo and Piergiorgio Modena: T Mastrangelo and P Modena contributed equally to this study

Authors and Affiliations

  1. Istituto Nazionale Tumori, via G, Venezian 1, 20133, Milano, Italy

    Tiziana Mastrangelo, Piergiorgio Modena, Silvana Tornielli, Maria Adele Testi, Alessandra Mezzelani, Paolo Radice, Alberto Azzarelli, Silvana Pilotti, Marco A Pierotti & Gabriella Sozzi

  2. Kimmel Cancer Center, Jefferson Medical College, Philadelphia, 19107, PA, USA

    Florentia Bullrich & Carlo M Croce

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  1. Tiziana Mastrangelo
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Mastrangelo, T., Modena, P., Tornielli, S. et al. A novel zinc finger gene is fused to EWS in small round cell tumor. Oncogene 19, 3799–3804 (2000). https://doi.org/10.1038/sj.onc.1203762

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