Mouse models of Desmoplastic Small Round Cell Tumor and

结缔组织增生小圆细胞瘤（DSRCT）是一种罕见但极具侵袭性的肿瘤，多发于青少年腹部。在迄今为止检查的所有DSRCT病例中，涉及尤因斯肉瘤基因（EWS）和Wilms肿瘤抑制基因（WT1）的染色体易位导致了一种新的转录因子的产生。另一个值得关注的儿童肿瘤是尤因肿瘤？其中EWS基因与Fli1基因融合。我们的实验室专注于了解EWS/WT1和EWS/Fli1基因产物的功能，以及它们如何在各自的癌症中促进肿瘤发生。

Desmoplastic small round cell tumor (DSRCT) is a rare but extremely aggressive cancer arising in young adolescents in the abdominal area. In all cases of DSRCT examined to date, a chromosomal translocation involving the Ewings Sarcoma gene (EWS) and the Wilms tumor suppressor gene, WT1, results in genesis of a novel transcription factor. Another pediatric tumor of interest is the Ewing?s sarcoma, where EWS gene is fused to the Fli1 gene. Our laboratory is focused on understanding the functions of EWS/WT1 and EWS/Fli1 gene products and how they contribute to tumorigenesis in their respective cancers. Towards this goal, we are currently developing mouse models of DSRCT and Ewing?s sarcoma to better understand the etiology and the development of these tumors in the context of whole organism. If successful, our animal models will be invaluable for the development of new and existing therapeutics against DSRCT and Ewing?s sarcoma.