EWS-fusion-protein
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Genetics
- EWS-fusion-proteins (EPFS) arise due to chromosomal translocations in which EWSR1 is fused to a variety of cellular transcription factors
- EFPS are very potent transcriptional activators dependent on the EAD & a C-terminal DNA-binding domain contributed by the fusion partner
- the spectrum of malignancies associated with EFPS are thought to arise via EFP-induced transcriptional deregulation, with tumor phenotype specified by the EWSR1 fusion partner & cell type
- transcriptional repression of the TGF beta-2 receptor is an important target of the EWS-FLI1, EWS-ERG, or EWS-ETV1 oncogene