肠神经系统(enteric nervous system,ENS)是机体内最大的外周神经系统,由神经元和胶质细胞组成。ENS在维持肠道正常功能中发挥重要作用,包括调控肠道的分泌、吸收及蠕动等。在发育过程中,ENS受到多条信号通路的调控,主要有GDNF-GFRα1-RET、NRTN-GFRα2-RET及EDN3-EDNRB3信号通路,同时,也受SOX10、Sprouty2、KIF26A等分子的调控。肠神经系统发育不良最常见的疾病是先天性巨结肠疾病,关于该疾病的治疗,有待于更进一步的探索。
The enteric nervous system (ENS) is the largest part of the peripheral nervous system and is an extensive network, composed of neurons and glia. The ENS plays a critical role in movement patterns of gastrointestinal tract, acid secretion and absorption. The development of ENS is regulated by multiple signaling pathways, including GDNF-GFRα1-RET, NRTN-GFRα2-RET and EDN3-EDNRB3. Furthermore, many additional molecules also play their role in the ENS development, such as SOXl0, Sprouty2, KIF26A and et al. Congenital megacolon (Hirschsprung disease, HSCR) is the major disease manifested by defects in the ENS development, and therapeutic treatments for this disease need to be actively searched.