神经嵴细胞导致主动脉弓发育不良的影响因素

Influential factors of hypoplastic aortic arch caused by neural crest cells

心脏神经嵴细胞(cardiac neural crest cells,CNCCs)是特殊的神经嵴细胞亚群,起源于外胚层神经管背侧,是胚胎发育过程中的多能干细胞群,可迁移到外周,分化成多种类型细胞,是心脏和流出道发育所必需.主动脉弓发育不良(hypoplastic aortic arch,HAA)是一类胚胎期心室流出道发育异常引起的先天性心脏畸形,且常合并其他心脏畸形,是复杂型先天性心脏病中较常见且出生后预后不良的一类疾病,严重危害儿童健康.CNCCs的发育异常与HAA有显著相关性,虽然目前其影响因素尚不明确,但相关实验已经筛选出有关的基因与环境因素.讨论其参与心脏流出道发育过程的机制,有助于对HAA发病机制的理解,进而推进治疗方法的进展.本文就影响CNCCs发育的基因及环境因素及其与HAA的相关性作一综述.

Cardi ac neural crest cells(CNCCs)are a special subgroup of neural crest cells that originate from the dorsal side of the ectodermal neural tube.During embryonic development,they are a pluripotent stem cell group that can migrate to the periphery and differentiate into many types of cells.They are essential for proper development of heart and outflow tract(OFT).Hypoplastic aortic arch(HAA)is a type of congenital heart malformation caused by abnormal development of the ventricular outflow tract in the embryonic period,and often combined with other cardiac malformations.It is a common and poor prognosis after birth in complex congenital heart disease,which seriously endangers children©health.There is a significant correlation between abnormal development of CNCCs and HAA.Although the influencing factors are still unclear,related experiments have screened out relevant genetic and environmental factors.Discussing the mechanism of its involvement in the development of cardiac outflow tract is helpful to understand the pathogenesis of HAA and promote the progress of treatment methods.This article reviews the genetic and environmental factors affecting the development of CNCCs and their correlation,with HAA.