Biology of EWS/ETS fusions in Ewing's family tumors.
Tumor-associated chromosomal translocations lead to the formation of chimeric fusions between the EWS gene and one of five different ETS transcription factors in Ewing's family tumors (EFTs). The resultant EWS/ETS proteins promote oncogenesis in a dominant fashion in model systems and are necessary for continued growth of EFT cell lines. EWS belongs to a family of genes that encode proteins that may serve as adapters between the RNA polymerase II complex and RNA splicing factors. EWS/ETS fusions have biochemical characteristics of aberrant transcription factors and appear to promote abnormal cellular growth by transcriptionally modulating a network of target genes. Early evidence suggests that EWS/ETS proteins may also impact gene expression through alteration in RNA processing. Elucidation of EWS/ETS target gene networks in the context of other signaling pathways will hopefully lead to biology based therapeutic strategies for EFT.
Arvand
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Department of Pathology and Laboratory Medicine, Gwynne Hazen Cherry Memorial Labs, University of California at Los Angeles, California, USA.
Denny
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D002455:Cell Division; D034441:Heterogeneous-Nuclear Ribonucleoproteins; D007621:Karyotyping; D008954:Models, Biological; D008957:Models, Genetic; D005810:Multigene Family; D009369:Neoplasms / Q000209:etiology / Q000235:genetics; D015514:Oncogene Proteins, Fusion / Q000235:genetics* / Q000378:metabolism*; D011485:Protein Binding; D017434:Protein Structure, Tertiary; D012313:RNA / Q000378:metabolism; D034802:RNA-Binding Protein EWS; D012261:Ribonucleoproteins / Q000737:chemistry* / Q000235:genetics / Q000378:metabolism; D012512:Sarcoma, Ewing / Q000235:genetics*; D014178:Translocation, Genetic
D034441:Heterogeneous-Nuclear Ribonucleoproteins; D015514:Oncogene Proteins, Fusion; D034802:RNA-Binding Protein EWS; D012261:Ribonucleoproteins; D012313:RNA