EWSR1是致癌基因，编码蛋白是高度保守的异质核核糖核蛋白颗粒（HNRNP）蛋白家族FET的三个成员中的一个，是一种RNA结合蛋白。EWSR1蛋白可与DNA或RNA结合，通过与RNA Pol II结合、RNA加工和代谢以及DNA损伤修复而在转录中起作用。EWSR1的N端结构域可融合到多种转录因子的C端结构域，在不同的软组织肿瘤中具有各种致癌基因-伴侣，在尤文氏肉瘤中常见的融合形式为EWSR1-FLI1。

EWSR1 is one of three members of the highly conserved heterogeneous nuclear ribonucleoprotein particle (hnRNP) protein family, FET . The EWSR1 protein can bind to DNA or RNA and is known to play a role in transcription via binding to RNA Pol II, RNA processing and metabolism, and DNA damage repair. The N-terminal domain of EWSR1 can fuse to the C-terminal domain of a number of transcription factors and give rise to several fusion proteins that have been implicated in Ewing Sarcoma, primitive neuroectodermal tumor, desmoplastic small round cell tumor, myxoid liposarcoma, extraskeletal myxoid chondrosarcoma, and clear cell sarcoma. The EWSR1-FLI1 fusion is known to be oncogenic.

GeneCards OncoKB My Cancer Genome