Molecular mechanism of neural crest cell proliferation, differentiation and death

神经嵴细胞分化成不同的组织类型，如肠神经元、交感神经元和黑素细胞。神经嵴细胞发育异常可导致先天性巨结肠、神经母细胞瘤等新生儿畸形综合征。我们已经鉴定了在神经嵴衍生细胞中特异性表达的Ncx，并寻找了其靶基因。我们发现，Nczf，靶基因之一，控制细胞增殖在胚胎发育过程中，负调控p27的表达。另一个靶基因Prickle 2调节神经母细胞瘤细胞系中的神经突延伸。对Ncx靶基因网络的分析将有助于利用ES或iPS细胞进行退行性医学治疗神经嵴病的新方法的开发。

Neural crest cells differentiate into diverse tissue types such as enteric neurons, sympathetic neurons, and melanocytes. Abnormal development of neural crest cells results in Hirschsprung's disease, neuroblastoma, and other anomaly syndrome in newborn. We have identified Ncx which is specifically expressed in neural crest derived cells and searched for its target genes. We found that Nczf, one of the target genes, controls cell proliferation during embryogenesis by negatively regulating p27 expression. Another target gene, Prickle 2, regulates neurite extention in neuroblastoma cell line. Analysis of Ncx target gene network will contribute to the development of new therapeutic methods of neurocristopathy by degenerative medicine using ES or iPS cells.