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A novel zinc finger gene is fused to EWS in small round cell tumor.
Oncogene. 2000 Aug 03; 19(33):3799-804.O

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.

Authors+Show Affiliations

Istituto Nazionale Tumori, Milano, Italy.No affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

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

Language

eng

PubMed ID

10949935

Citation

Mastrangelo, T, et al. "A Novel Zinc Finger Gene Is Fused to EWS in Small Round Cell Tumor." Oncogene, vol. 19, no. 33, 2000, pp. 3799-804.
Mastrangelo T, Modena P, Tornielli S, et al. A novel zinc finger gene is fused to EWS in small round cell tumor. Oncogene. 2000;19(33):3799-804.
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), 3799-804.
Mastrangelo T, et al. A Novel Zinc Finger Gene Is Fused to EWS in Small Round Cell Tumor. Oncogene. 2000 Aug 3;19(33):3799-804. PubMed PMID: 10949935.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - A novel zinc finger gene is fused to EWS in small round cell tumor. AU - Mastrangelo,T, AU - Modena,P, AU - Tornielli,S, AU - Bullrich,F, AU - Testi,M A, AU - Mezzelani,A, AU - Radice,P, AU - Azzarelli,A, AU - Pilotti,S, AU - Croce,C M, AU - Pierotti,M A, AU - Sozzi,G, PY - 2000/8/19/pubmed PY - 2000/9/9/medline PY - 2000/8/19/entrez SP - 3799 EP - 804 JF - Oncogene JO - Oncogene VL - 19 IS - 33 N2 - 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. SN - 0950-9232 UR - https://www.unboundmedicine.com/medline/citation/10949935/A_novel_zinc_finger_gene_is_fused_to_EWS_in_small_round_cell_tumor_ L2 - https://doi.org/10.1038/sj.onc.1203762 DB - PRIME DP - Unbound Medicine ER -