神经嵴细胞迁移障碍在先天性巨结肠发病中的作用研究进展  

Research advances on the role of neural crest cell migration disorder in Hirschsprung disease

作　　者：翟威 刘远梅 Zhai Wei;Liu Yuanmei(Department of Pediatric Surgery,Affiliated Hospital,Zunyi Medical University,Zunyi 563000,China)

机构地区：[1]遵义医科大学附属医院小儿外科,遵义563000

出　　处：《中华小儿外科杂志》2023年第1期87-91,共5页Chinese Journal of Pediatric Surgery

基　　金：国家自然科学基金(82060100)。

摘　　要：神经嵴细胞起源于神经和非神经外胚层的交界处(神经板交界处),具有显著的多能性和迁移能力,在胚胎中广泛迁移至最终目的地,分化成相应组织或器官。先天性巨结肠(Hirschsprung disease,HSCR)是由于胚胎早期神经嵴细胞迁移障碍,导致胚胎期肠管缺乏足够数量的肠神经嵴细胞定植,因而导致肠神经系统形成障碍,而引起患儿腹胀便秘等症状的先天性消化道畸形。因此,HSCR的发病与神经嵴细胞迁移异常密切相关。现就细胞外基质的降解和重构、神经嵴细胞上皮-间质转化以及细胞间突起形成影响神经嵴细胞迁移的研究现状进行综述。Derived from the junction of nerve and non-neural ectoderm(neural plate junction),neural crest cells(NCC)possess significant capacities of pluripotency and migration,migrate extensively to their final destination in embryo and differentiate into corresponding tissues or organs.Congenital Hirschsprung's disease(HD)is caused by a lack of entric neural crest cells(ENCC)colonization in embryonic intestine due to an early embryonic NCC migration disorder.As a result,intestinal nervous system disorders cause abdominal distention and constipation symptoms of congenital gastrointestinal malformation.Thus the pathogenesis of HD is closely correlated with abnormal NCC migration.Degradation and remodeling of extracellular matrix(ECM),epithelial-mesenchymal transformation(EMT)and formation of intercellular processes affect NCC migration.

关 键 词：先天性巨结肠 神经嵴细胞 细胞外基质 上皮-间质转化 迁移 

分 类 号：R725.7[医药卫生—儿科]