EWSR1-FLI1 fusion protein (EWS::FLI1 (or EWS-FLI1))

The EWSR1-FLI1 fusion protein (also denoted EWS::FLI1 or EWS-FLI1) is an oncogenic fusion oncoprotein created by a chromosomal translocation fusing the N-terminal low-complexity domain (LCD) and transactivation domain of the RNA-binding protein EWSR1 (Ewing sarcoma breakpoint region 1) with the C-terminal ETS DNA-binding domain of the transcription factor FLI1 (Friend leukemia integration 1); it drives over 85% of Ewing sarcoma tumors by acting as an aberrant transcription factor that reprograms enhancers at GGAA microsatellites, promotes phase separation into biomolecular condensates, interferes with wild-type EWSR1 functions via dominant-negative mechanisms, alters RNA splicing and stability, disrupts DNA damage repair, and regulates cell proliferation and transformation.

Other names

EWS-FLI1EWS::FLI1EWSR1::FLI1EWS/FLI1EWSR1/FLI1