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Identification of genes regulated by the EWS/NR4A3 fusion protein in extraskeletal myxoid chondrosarcoma

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Tumor Biology

Abstract

Approximately 75 % of extraskeletal myxoid chondrosarcoma tumors (EMC) harbor a t(9;22) chromosome translocation generating an EWS/NR4A3 fusion protein that is thought to be instrumental in the tumoral process. Current evidence suggests that one function of the fusion protein is to overexpress target genes. We have generated an in vitro human cellular model in which the fusion protein is expressed in mesenchymal bone marrow stem cells. We have performed microarray analyses of these cells and identified several genes overexpressed in the presence of EWS/NR4A3 which are also overexpressed in EMC tumors. These genes and their products represent potential therapeutic targets for EMC tumors.

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Acknowledgments

The authors thank Drs. Marc Ladanyi, Memorial Sloan-Kettering Cancer Center, and Bernard Têtu, Université Laval, for tumor samples. This work was supported by a grant from the Sarcoma Foundation of America (SFA10-11).

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Authors and Affiliations

  1. Centre de recherche de l’Hôpital Saint-François d’Assise/CHUQ, 10 rue de l’Espinay, local D0-711, Québec, QC, Canada, G1L 3L5

    Christine Filion & Yves Labelle

  2. Faculté de médecine de l’Université Laval, Québec, QC, Canada, G1V 0A6

    Yves Labelle

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  1. Christine Filion
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  2. Yves Labelle
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Correspondence to Yves Labelle.

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Filion, C., Labelle, Y. Identification of genes regulated by the EWS/NR4A3 fusion protein in extraskeletal myxoid chondrosarcoma. Tumor Biol. 33, 1599–1605 (2012). https://doi.org/10.1007/s13277-012-0415-2

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  • DOI: https://doi.org/10.1007/s13277-012-0415-2

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