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Beschreibung

RNA-binding protein EWS (UniProt: Q01844, also known as EWS oncogene, Ewing sarcoma breakpoint region 1 protein) is encoded by the EWSR1 (also known as EWS) gene (Gene ID: 2130) in human. EWS is member of the TET family of DNA and RNA-binding proteins, which also includes translocated in liposarcoma/fused in sarcoma protein (FUS/TLS) and TATA-binding protein-associated factor 15. It is a multifaceted RNA binding protein with established roles in transcription, pre-mRNA processing, and DNA damage response. It contains a transcriptional-activation domain (EAD), 3 glycine-arginine rich regions, an RNA-binding domain, and a zinc finger domain (aa 518-549). EWS is highly methylated on arginine residues and methylation is mediated by PRMT1 and to some extent by PRMT8. Various environmental signals are known to induce post-translational modifications in its RNA binding domain and glycine-arginine rich domains, thus modulating EWS activity. EWS can relocate from cytoplasm to ribosomes upon PTK2B/FAK2 activation. Mutations in EWS gene are known to cause Ewing sarcoma, a highly malignant, metastatic, primitive small round cell tumor of bone and soft tissue.