RNA-binding protein EWS (also known as EWSR1) participates in transcriptional regulation and RNA processing. It is structurally and functionally related to FUS (TLS) protein []. EWSR1 gene rearrangements are often observed in myoepithelial tumours [, ].TAF15/EWS/TLS family members include human FUS (Fused in liposarcoma), EWS (Ewing Sarcoma) and TAF15 (TATA binding associated factor 15), and the Drosophila orthologue Cabeza. They are RNA binding proteins that contain a transcriptional-activation domain (EAD), 3 glycine-arginine (RGG) rich regions, an RNA-binding domain (RBD), and a zinc finger domain [, ]. They are involved in transcription and alternative splicing. They are subjected to different environmental signals that induce post-translational modifications in their RBD and in the RGG domains, thus modulating their activity []. TAF15/EWS/TLS play important roles in oncogenesis and neuronal disease [].
This entry represents the RNA recognition motif (RRM) of EWS.RNA-binding protein EWS (also known as EWSR1) participates in transcriptional regulation and RNA processing. It is structurally and functionally related to FUS (TLS) protein []. EWSR1 gene rearrangements are often observed in myoepithelial tumours [, ].
Rhomboid domain-containing protein 3 (RHBDD3) is encoded by chromosome 22 CpG island-associated gene (C22orf3) that is not expressed in a significant proportion of pituitary tumours. C22orf3 is also called pituitary tumour apoptosis gene (PTAG) or RHBDD3, and is located directly upstream of EWSR1 []. Although its biological function remains unclear, RHBDD3 contains an N-terminal rhomboid domain and a C-terminal ubiquitin-associated (UBA) domain. RHBDD3 is wide expressed in immune cells and in NK cells it negatively regulates TLR3-mediated activation [].
