Establishment of mouse model of synovial sarcomas using lineage-specific gene targeting system

为了验证滑膜肉瘤起源于神经脊细胞的假说，我们建立了以P0基因为驱动力，在神经脊细胞中诱导表达SS特异性融合基因SYT-SSX的转基因小鼠。融合基因在子宫内的表达抑制了颅面骨和软骨组织的发育，导致死产。然后利用三苯氧胺-ER系统建立了可诱导的转基因小鼠。用他莫昔芬处理后，在P0阳性细胞中成功地诱导了SYT-SSX及其下游基因的表达，表明该小鼠模型是了解滑膜肉瘤发生发展的有用工具。

To prove out hypothesis that cell-of-origin of synovial sarcomas(SS) is neural crest derived cells, we established transgenic mice in which the expression of SS-specific fusion gene, SYT-SSX was induced in neural crest cells using the P0 gene as a driver. The expression of fusion gene in utero inhibited the development of craniofacial bone and cartilage tissues causing stillbirth. Then we established inducible transgenic mice using tamoxifen-ER system. Treatment with tamoxifen successfully induced the expression of SYT-SSX and also its downstream gene in P0 positive cells, indicating that this mouse model is a useful tool to understand the development of synovial sarcomas.