·综述·心脏发育的信号调控与先天性心脏病的关系.pdfVIP

　 军 杂 2013年11月1 日　第38卷　第11期 952 解放 医学 志　 ·综　　述· 心脏发育的信号调控与先天性心脏病的关系 王力，汤楚中，朱智明 [摘要]　心脏发育起源于原肠胚阶段位于前侧板中胚层的生心区，生心区祖细胞在特异细胞因子、诱导信号及核 心转录因子构成的调控网络作用下分化为心肌前体细胞，心脏发育过程中的基因变异或发育环境变化可影响调控网络 中的多个环节，最后导致先天性心脏病的发生。因此，研究心脏发育过程中的信号调控机制对于探讨先天性心脏病 的发生机制具有重要的理论及临床意义。目前研究证实Nkx2.5 、GATA4 、Tbx5 、Isl-1等众多早期转录因子均参与了心 脏发育的基因调控网络，但网络中大部分信号通路的研究尚不深入。本文就目前研究较多的心肌前体细胞、Nkx2.5 、 GATA4、Tbx5 、Isl-1等转录因子以及Apelin/APJ信号通路的近期研究进展作一简要综述。 [关键词]　心脏；胚胎发育；先天性心脏病；信号转导；转录因子 [中图分类号]　R346　　　　[文献标志码]　A　　　　[文章编号]　0577-7402(2013)11-0952-05 [DOI]　10.11855/j.issn.0577-7402.2013.11.019 Relationship between regulation of signaling pathways in heart development and congenital heart diseases 1 2 2* WANG Li , TANG Chu-zhong , ZHU Zhi-ming 1Clinical College of Navy Medicine, Second Military Medical University, Beijing 100048, China 2Department of Cardiology, Navy General Hospital, Beijing 100048, China * Corresponding author, E-mail: zhuzhiming6542@ This work was supported by the National Natural Science Foundation of China [Abstract] 　The cardiac lineage arises from the cardiogenic area located in anterior lateral plate mesoderm during gastrula stage. Differentiation of cells in cardiac mesoderm into cardiac progenitor cells was regulated by complex signaling networks involving specific cytokines, inducing signal proteins and the core cardiac transcription factors. Any link of signaling networks influenced by mutation in genetics and changes in environment would lead to a series of congenital heart defects. Therefore, studies of signal transduction mechanism in heart development will give rise to a series of significant theoretical and clinical contributions to the mechanism of development of congenital heart diseases. Recently, it has been proved that a group of early transcri