中文摘要：

背景 Tbxl 是为 DiGeorge 症候群(DGS ) 的主要候选人基因。类似于在 DGS 病人，观察的缺点在 Tbxl-/- 动物模型破坏的结构在开发期间从神经的冠房间被导出。尽管一些 Tbxl 击倒的动物模型的词法显型是描述的井，心脏的表演的分析是有限的。因此，心肌的性能在 Tbxl morpholino 被探索注射 zebrafish 胚胎。阐明的方法这些问题， Tbxl 特定的 morpholino 被用来在 zebrafish 减少 Tbxl 的功能。心肌的房间的区别在 situ 杂交用整个山被观察。心率从 24 ～ 72 小时柱子授精(hpf ) 在显微镜下面被观察并且记录。心脏的表演被测量室的弄短的部分和 atrial 弄短 fraction.Results Tbxl morpholino 分析注射胚胎被缺点在咽头的拱门，耳的泡，大动脉的拱门和胸腺描绘。另外， Tbxl 敲击在下面在 zebrafish 减少了咽头的神经的冠房间的数量。反常心脏的形态学在将近 20% Tbxl morpholino 是可见的注射胚胎。在这些胚胎的心没不完全地循环或循环。重要地，心脏的性能和心率在注射 embryos.Conclusions Tbxl 可能玩的 Tbxl morpholino 被减少在在 zebrafish 的咽头的神经的冠房间的开发的一个必要角色。心脏的性能被 Tbxl 损害在 zebrafish 击倒。

英文摘要：

Background Tbxl is the major candidate gene for DiGeorge syndrome （DGS）. Similar to defects observed in DGS patients, the structures disrupted in Tbxl-/- animal models are derived from the neural crest cells during development. Although the morphological phenotypes of some Tbxl knock-down animal models have been well described, analysis of the cardiac performance is limited. Therefore, myocardial performance was explored in Tbxl morpholino injected zebrafish embryos. Methods To elucidate these issues, Tbxl specific morpholino was used to reduce the function of Tbxl in zebrafish. The differentiation of the myocardial cells was observed using whole mount in situ hybridization. Heart rates were observed and recorded under the microscope from 24 to 72 hours post fertilization （hpf）. The cardiac performance was analyzed by measuring ventricular shortening fraction and atrial shortening fraction.Results Tbxl morpholino injected embryos were characterized by defects in the pharyngeal arches, otic vesicle, aortic arches and thymus. In addition, Tbxl knock down reduced the amount of pharyngeal neural crest cells in zebrafish. Abnormal cardiac morphology was visible in nearly 20% of the Tbxl morpholino injected embryos. The hearts in these embryos did not loop or loop incompletely. Importantly, cardiac performance and heart rate were reduced in Tbxl morpholino injected embryos.Conclusions Tbxl might play an essential role in the development of pharyngeal neural crest cells in zebrafish. Cardiac performance is impaired by Tbxl knock down in zebrafish.