Ewing sarcoma is a cancer of bone or its surrounding soft tissue that primarily affects children and young adults.
A hallmark of Ewing sarcoma is a translocation event that results in the fusion of an RNA binding protein, known as EWS, with a transcription factor, such as FLI1.
Previous work suggested that the fusion protein EWS-FLI1 promoted cancer by changing gene expression; however, the gene targets were unknown.
A new study in the Journal of Clinical Investigation indicates that a long noncoding RNA named Ewing sarcoma-associated transcript 1 (EWSAT1) is a critical target of the fusion protein and contributes to the complex network of changes that occur in Ewing Sarcoma.
A team led by Alejandro Sweet-Cordero at Stanford University identified increased expression of EWSAT1 in cancer cells from children with Ewing sarcoma.
Further they showed that this noncoding RNA is important for cancer cell growth and associated with the repression of several genes downstream of EWS-FLI1.
Their work supports the notion that long noncoding RNAs can be key drivers of cancer and identifies an important mediator of Ewing Sarcoma.
Long noncoding RNA EWSAT1-mediated gene repression facilitates Ewing sarcoma oncogenesis J Clin Invest. 2014;124(12):5275–5290. doi:10.1172/JCI72124.