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先天性心脏病中的平面细胞极性信号通路。

Planar cell polarity signaling pathway in congenital heart diseases.

作者信息

Wu Gang, Ge Jiao, Huang Xupei, Hua Yimin, Mu Dezhi

机构信息

Department of Pediatrics, West China Second University Hospital, Sichuan University, Chengdu, Sichuan 610041, China.

出版信息

J Biomed Biotechnol. 2011;2011:589414. doi: 10.1155/2011/589414. Epub 2011 Oct 29.

Abstract

Congenital heart disease (CHD) is a common cardiac disorder in humans. Despite many advances in the understanding of CHD and the identification of many associated genes, the fundamental etiology for the majority of cases remains unclear. The planar cell polarity (PCP) signaling pathway, responsible for tissue polarity in Drosophila and gastrulation movements and cardiogenesis in vertebrates, has been shown to play multiple roles during cardiac differentiation and development. The disrupted function of PCP signaling is connected to some CHDs. Here, we summarize our current understanding of how PCP factors affect the pathogenesis of CHD.

摘要

先天性心脏病(CHD)是人类常见的心脏疾病。尽管在CHD的认识以及许多相关基因的鉴定方面取得了诸多进展,但大多数病例的根本病因仍不清楚。平面细胞极性(PCP)信号通路负责果蝇的组织极性、脊椎动物的原肠胚形成运动和心脏发生,已被证明在心脏分化和发育过程中发挥多种作用。PCP信号功能的破坏与某些CHD有关。在这里,我们总结了目前对PCP因子如何影响CHD发病机制的理解。

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