Desmoplastic Small Round Cell Tumor/Ewings Sarcoma Model

促纤维增生性小圆细胞肿瘤/尤文氏肉瘤模型

• 批准号: 7153399
• 负责人: Sean B. Lee
• 金额: --
• 依托单位: NATIONAL INSTITUTE OF DIABETES AND DIGESTIVE AND KIDNEY DISEASES
• 依托单位国家: 美国
• 资助国家: 美国
• 起止时间: 至
• 项目状态: 未结题
• 来源: https://reporter.nih.gov/project-details/7153399
• 关键词: Ewing' s tumor; abdomen neoplasm; chromosome translocation; disease /disorder etiology; disease /disorder model; gene mutation; genetically modified animals; human tissue; laboratory mouse; model design /development; neoplasm /cancer genetics; pathologic process; pediatric neoplasm /cancer; sarcoma; transcription factor; tumor suppressor genes

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.

促结缔组织增生性小圆细胞肿瘤（DSRCT）是一种罕见的，但非常积极的癌症发生在年轻的青少年在腹部地区。在迄今为止检查的所有DSRCT病例中，涉及尤因斯肉瘤基因（EWS）和威尔姆斯肿瘤抑制基因WT 1的染色体易位导致了一种新型转录因子的产生。另一个儿童肿瘤是尤因？S肉瘤，其中EWS基因与Fli 1基因融合。我们的实验室专注于了解EWS/WT 1和EWS/Fli 1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。 为了实现这一目标，我们目前正在开发小鼠模型DSRCT和尤因？的肉瘤，以更好地了解病因和发展，这些肿瘤的背景下，整个有机体。如果成功，我们的动物模型将是非常宝贵的新的和现有的治疗方法的发展对DSRCT和尤因？s肉瘤。