Definition
The EWS-FLI1 fusion transcript is the result of a balanced reciprocal chromosomal translocation between chromosomes 11 and 22, which fuses the EWS gene in chromosome 22 to the FLI1 gene in chromosome 11. This fusion transcript is detected in approximately 85 % of cases of the Ewing sarcoma family of tumors and is considered a tumor-specific molecular rearrangement, therefore useful for diagnosis, prognosis, and presumably specific therapeutics. Approximately 10 % of Ewing tumors have fusions involving EWS and ERG genes. An additional 5 % bear fusions between EWS and other less frequent genes.
Characteristics
Structure
Chromosomal translocations result in the genesis of chimeric genes, encoding hybrid transcripts and novel fusion proteins. Many fusion proteins contain juxtaposed functional domains usually found in separate proteins. The EWS-FLI1 fusion protein contains the aminoterminal domain of EWS and the carboxyterminal region of FLI1. EWSgene is an RNA-binding protein,...
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References
Alava E, de Gerald WL (2000) Molecular biology of the Ewing’s sarcoma/primitive neuroectodermal tumor family. J Clin Oncol 18:204–213
Hu-Lieskovan S, Heidel JD, Bartlett DW et al (2005) Sequence-specific knockdown of EWS-FLI1 by targeted, nonviral delivery of small interfering RNA inhibits tumor growth in a murine model of metastatic Ewing’s sarcoma. Cancer Res 65:8984–8992
Kovar H (2005) Context matters: the hen or egg problem in Ewing’s sarcoma. Semin Cancer Biol 15:189–196
Riggi N, Suva ML, Stamenkovic I (2006) Ewing’s Sarcoma-like tumors originate from EWS-FLI-1-expressing mesenchymal progenitor cells. Cancer Res 66:9786
Schleiermacher G, Peter M, Oberlin O et al (2003) Increased risk of systemic relapses associated with bone marrow micrometastasis and circulating tumor cells in localized Ewing tumor. J Clin Oncol 21:85–91
See Also
(2012) CD99. In: Schwab M (ed) Encyclopedia of cancer, 3rd edn. Springer, Berlin/Heidelberg, p 704. doi:10.1007/978-3-642-16483-5_941
(2012) Chromosomal translocation t(9;22). In: Schwab M (ed) Encyclopedia of cancer, 3rd edn. Springer, Berlin/Heidelberg, pp 845–846. doi:10.1007/978-3-642-16483-5_1142
(2012) Formalin-fixed Paraffin-embedded Tissue. In: Schwab M (ed) Encyclopedia of cancer, 3rd edn. Springer, Berlin/Heidelberg, p 1446. doi:10.1007/978-3-642-16483-5_2249
(2012) Illegitimate recombination. In: Schwab M (ed) Encyclopedia of cancer, 3rd edn. Springer, Berlin/Heidelberg, p 1808. doi:10.1007/978-3-642-16483-5_2960
(2012) RT-PCR. In: Schwab M (ed) Encyclopedia of cancer, 3rd edn. Springer, Berlin/Heidelberg, p 3322. doi:10.1007/978-3-642-16483-5_5129
(2012) Transcript. In: Schwab M (ed) Encyclopedia of cancer, 3rd edn. Springer, Berlin/Heidelberg, p 3752. doi:10.1007/978-3-642-16483-5_5898
(2012) Transcription factor. In: Schwab M (ed) Encyclopedia of cancer, 3rd edn. Springer, Berlin/Heidelberg, p 3752. doi:10.1007/978-3-642-16483-5_5901
(2012) Translocation. In: Schwab M (ed) Encyclopedia of cancer, 3rd edn. Springer, Berlin/Heidelberg, p 3773. doi:10.1007/978-3-642-16483-5_5942
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de Alava, E., y Cajal, S.R. (2015). EWS-FLI (ets) Fusion Transcripts. In: Schwab, M. (eds) Encyclopedia of Cancer. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-642-27841-9_2046-3
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DOI: https://doi.org/10.1007/978-3-642-27841-9_2046-3
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Publisher Name: Springer, Berlin, Heidelberg
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