Department of Pathology and Cell Biology, Columbia University, College of Physicians and Surgeons, 630 West 168th Street, New York, NY 10032, USA.
Trends Neurosci. 2010 Oct;33(10):446-56. doi: 10.1016/j.tins.2010.06.002. Epub 2010 Jul 13.
Enteric nervous system (ENS) development is relevant to Hirschsprung's disease (HSCR; congenital aganglionosis of the terminal bowel), which is still imperfectly treated. Mutations in genes encoding the RET receptor tyrosine kinase and endothelin receptor type B (EDNRB) are involved in HSCR pathogenesis; however, also important in ENS development are molecules that mediate events that are more restricted than those of RET and EDNRB, act later in development and which might not be HSCR-associated. Examples are molecules that function in the guidance of enteric neural crest-derived cells (ENCDCs) and vagal axons, and in regulating the terminal differentiation of enteric neurons from ENCDCs. It is probable that highly prevalent disorders of gastrointestinal sensation and motility result from subtle defects in ENS development.
肠神经系统（ENS）的发育与先天性巨结肠（HSCR；末端肠无神经节）有关，后者的治疗仍不完善。编码 RET 受体酪氨酸激酶和内皮素受体 B（EDNRB）的基因突变参与了 HSCR 的发病机制；然而，在 ENS 发育中起重要作用的还有那些介导比 RET 和 EDNRB 更局限的事件的分子，它们在发育后期发挥作用，并且可能与 HSCR 无关。例如，在肠神经嵴源性细胞（ENCDCs）和迷走神经轴突的导向以及调节 ENCDCs 来源的肠神经元的终末分化中发挥作用的分子。很可能胃肠道感觉和运动的高发紊乱是由于 ENS 发育的细微缺陷所致。
