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Biology of EWS/ETS fusions in Ewing s family tumors Arvand A; Denny CTOncogene 2001[Sep]; 20 (40): 5747-54Tumor-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.|Cell Division[MESH]|Heterogeneous-Nuclear Ribonucleoproteins[MESH]|Karyotyping[MESH]|Models, Biological[MESH]|Models, Genetic[MESH]|Multigene Family[MESH]|Neoplasms/etiology/genetics[MESH]|Oncogene Proteins, Fusion/*genetics/*metabolism[MESH]|Protein Binding[MESH]|Protein Structure, Tertiary[MESH]|RNA-Binding Protein EWS[MESH]|RNA/metabolism[MESH]|Ribonucleoproteins/*chemistry/genetics/metabolism[MESH]|Sarcoma, Ewing/*genetics[MESH]|Translocation, Genetic[MESH] |
