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Sarcoma
Volume 2011, Article ID 352580, 11 pages
http://dx.doi.org/10.1155/2011/352580
Review Article

Factors Affecting EWS-FLI1 Activity in Ewing's Sarcoma

Children's Cancer Research Institute, St Anna Kinderkrebsforschung, 1090 Vienna, Austria

Received 15 July 2011; Revised 31 August 2011; Accepted 31 August 2011

Academic Editor: Alessandro Gronchi

Copyright © 2011 David Herrero-Martin et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

Ewing's sarcoma family tumors (ESFT) are characterized by specific chromosomal translocations, which give rise to EWS-ETS chimeric proteins. These aberrant transcription factors are the main pathogenic drivers of ESFT. Elucidation of the factors influencing EWS-ETS expression and/or activity will guide the development of novel therapeutic agents against this fatal disease.