...
首页> 外文期刊>Trends in Cardiovascular Medicine >The role of Pitx2 during cardiac development. Linking left-right signaling and congenital heart diseases.
【24h】

The role of Pitx2 during cardiac development. Linking left-right signaling and congenital heart diseases.

机译:Pitx2在心脏发育过程中的作用。将左右信号与先天性心脏病联系起来。

获取原文
获取原文并翻译 | 示例
           

摘要

Pitx2 is a bicoid-related homeodomain transcription factor that plays a critical role in directing cardiac asymmetric morphogenesis. Ectopic Pitx2c expression in the developing myocardium correlates with double outlet right ventricle (DORV) in laterality mutants. Pitx2 loss of function experiments cause severe cardiovascular defects, such as atrial isomerism (AI), double inlet left ventricle, transposition of the great arteries (TGA), persistent truncus arteriosus (PTA), and abnormal aortic arch (AAA) remodeling. Current studies suggest that Pitx2-mediated signaling during cardiogenesis is conducted within three different cell types: the myocardium, the cardiac neural crest (CNC) cells, and the pharyngeal arch mesenchyme. Impaired Pitx2 function in discrete myocardial regions seems to lead to DORV, AI, and possibly TGA. On the other hand, impaired Pitx2 expression in the CNC leads preferentially to PTA. AAA remodeling is likely to occur owing to impaired cross-talk of the CNC cells with the pharyngeal arch mesenchyme. Thus, Pitx2 appears to be directing left-right identity to the cardiac venous components (e.g., the atria), whereas it appears to be modeling the morphologic arrangement of distinct myocardial components in the arterial pole. These data suggest that altered left-right signaling underlies the etiology of several common congenital cardiac malformations.
机译:Pitx2是与二倍体相关的同源域转录因子,在指导心脏不对称形态发生中起关键作用。在发育中的心肌中异位Pitx2c表达与侧向突变体中的双出口右心室(DORV)相关。 Pitx2功能丧失实验会导致严重的心血管缺陷,例如心房异构(AI),左心室双入口,大动脉移位(TGA),持续性动脉瘤(PTA)和主动脉弓异常(AAA)重塑。当前的研究表明,在心脏发生过程中Pitx2介导的信号传导是在三种不同的细胞类型中进行的:心肌,心脏神经neural(CNC)细胞和咽弓间充质。离散心肌区域Pitx2功能受损似乎会导致DORV,AI以及可能的TGA。另一方面,CNC中Pitx2表达受损会优先导致PTA。由于CNC细胞与咽弓间充质的串扰受损,很可能发生AAA重塑。因此,Pitx2似乎将左右同一性引导至心脏静脉组件(例如心房),而它似乎正在模拟动脉极中不同心肌组件的形态排列。这些数据表明左右信号的改变是一些常见的先天性心脏畸形的病因学基础。

著录项

相似文献

  • 外文文献
  • 中文文献
  • 专利
获取原文

客服邮箱:kefu@zhangqiaokeyan.com

京公网安备:11010802029741号 ICP备案号:京ICP备15016152号-6 六维联合信息科技 (北京) 有限公司©版权所有
  • 客服微信

  • 服务号