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| COMBINATORIAL GENERATION OF VARIABLE FUSION PROTEINS IN THE EWING FAMILY OF TUMORS |
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| Author(s): ZUCMAN J, MELOT T, DESMAZE C, GHYSDAEL J, PLOUGASTEL B, PETER M, ZUCKER JM, TRICHE TJ, SHEER D, TURCCAREL C, AMBROS P, COMBARET V, LENOIR G, AURIAS A, THOMAS G, DELATTRE O |
| Source: EMBO JOURNAL Volume: 12 Issue: 12 Pages: 4481-4487 Published: DEC 1 1993 |
| Times Cited: 381 References: 26 |
| Abstract: Balanced translocations involving band q12 of human chromosome 22 are the most frequent recurrent translocations observed in human solid tumours. It has been shown recently that this region encodes EWS, a protein with an RNA binding homologous domain. In Ewing's sarcoma and malignant melanoma of soft parts, translocations of band 22q12 to chromosome 11 and 12 result in the fusion of EWS with the transcription factors FLI-1 and ATF1, respectively. The present analysis of 89 Ewing's sarcomas and related tumours show that in addition to the expected EWS-FLI-1 fusion, the EWS gene can be fused to ERG, a transcription factor closely related to FLI-1 but located on chromosome 21. The position of the chromosome translocation breakpoints are shown to be restricted to introns 7-10 of the EWS gene and widely dispersed within introns 3-9 of the Ets-related genes. This heterogeneity generates a variety of chimeric proteins that can be detected by immunoprecipitation. On rare occasions, they may be associated with a truncated EWS protein arising from alternate splicing. All 13 different fusion proteins that were evidenced contained the N-terminal domain of EWS and the Ets domain of FLI-1 or ERG suggesting that oncogenic conversion is achieved by the linking of the two domains with no marked constraint on the connecting peptide. |
| Document Type: Article |
| Language: English |
Addresses:
1. INSERM, CJF 9201, GENET TUMEURS LAB, F-75231 PARIS 05, FRANCE
2. INST CURIE, SERV ONCOL PEDIAT, F-75231 PARIS 05, FRANCE
3. INST CURIE, ONCOGENESE VIRAL & CELLULAIRE LAB, URA D1443, F-91405 ORSAY, FRANCE
4. CHILDRENS HOSP, DEPT PATHOL & LAB MED, LOS ANGELES, CA 90027 USA
5. ICRF, HUMAN CYTOGENET LAB, LONDON WC2A 3PX, ENGLAND
6. CNRS, CYTOGENET CANCEROL LAB, URA 1462, F-06034 NICE, FRANCE
7. ST ANNA CHILDRENS HOSP, CCRI, A-1090 VIENNA, AUSTRIA
8. CTR LEON BERARD, IMMUNOL LAB, F-69373 LYON 08, FRANCE
9. CTR INT RECH CANC, F-69372 LYON 08, FRANCE |
| Publisher: OXFORD UNIV PRESS UNITED KINGDOM, WALTON ST JOURNALS DEPT, OXFORD, ENGLAND OX2 6DP |
| Subject Category: Biochemistry & Molecular Biology; Cell Biology |
| IDS Number: MF994 |
| ISSN: 0261-4189 |
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| | | | | | | | | Record from Web of Science® | | | | | | | | | |