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- Title
A novel zinc finger gene is fused to EWS in small round cell tumor.
- Authors
Mastrangelo, Tiziana; Modena, Piergiorgio; Tornielli, Silvana; Bullrich, Florentia; Testi, Maria Adele; Mezzelani, Alessandra; Radice, Paolo; Azzarelli, Alberto; Pilotti, Silvana; Croce, Carlo M; Pierotti, Marco A; Sozzi, Gabriella
- 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. Oncogene (2000) 19, 3799–3804
- Subjects
SARCOMA; FUSION (Phase transformation); ZINC-finger proteins
- Publication
Oncogene, 2000, Vol 19, Issue 33, p3799
- ISSN
0950-9232
- Publication type
Article
- DOI
10.1038/sj.onc.1203762