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

Oncogene. 2000 Aug 3;19(33):3799-804. doi: 10.1038/sj.onc.1203762.


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.

Publication types

  • Case Reports
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adolescent
  • Amino Acid Sequence
  • Base Sequence
  • Chromosomes, Human, Pair 1
  • Chromosomes, Human, Pair 22
  • DNA, Neoplasm
  • DNA-Binding Proteins
  • Humans
  • Kruppel-Like Transcription Factors
  • Lung Neoplasms / genetics*
  • Lung Neoplasms / secondary
  • Male
  • Molecular Sequence Data
  • Neoplasm Proteins / genetics*
  • Oncogene Proteins, Fusion / genetics*
  • Repressor Proteins*
  • Sarcoma, Ewing / genetics*
  • Sarcoma, Ewing / secondary
  • Translocation, Genetic
  • Zinc Fingers*


  • DNA, Neoplasm
  • DNA-Binding Proteins
  • Kruppel-Like Transcription Factors
  • Neoplasm Proteins
  • Oncogene Proteins, Fusion
  • PATZ1 protein, human
  • Repressor Proteins

Associated data

  • GENBANK/AF254082
  • GENBANK/AF254083
  • GENBANK/AF254084
  • GENBANK/AF254085
  • GENBANK/AF254086
  • GENBANK/AF254087
  • GENBANK/AF254088