Department of Pediatric Surgery, Qilu Hospital, Shandong University, 107 Wenhuaxi Road, Jinan 250012, Shandong, China.
Mol Biol Rep. 2013 Apr;40(4):2969-75. doi: 10.1007/s11033-012-2368-3. Epub 2012 Dec 23.
To investigate the expression levels of neurexins and neuroligins in the enteric nervous system (ENS) in Hirschsprung Disease (HSCR). Longitudinal muscles with adherent mesenteric plexus were obtained by dissection of the fresh gut wall of mice, guinea pigs, and humans. Double labeling of neurexin I and Hu (a neuron marker), neuroligin 1 and Hu, neurexin I and synaptophysin (a presynaptic marker), and neuroligin 1 and PSD95 (a postsynaptic marker) was performed by immunofluorescence staining. Images were merged to determine the relative localizations of the proteins. Expression levels of neurexin and neuroligin in different segments of the ENS in HSCR were investigated by immunohistochemistry. Neurexin and neuroligin were detected in the mesenteric plexus of mice, guinea pigs, and humans with HSCR. Neurexin was located in the presynapse, whereas neuroligin was located in the postsynapse. Expression levels of neurexin and neuroligin were significant in the ganglionic colonic segment of HSCR, moderate in the transitional segment, and negative in the aganglionic colonic segment. The expressions of neurexin and neuroligin in the transitional segments were significantly down-regulated compared with the levels in the normal segments (P < 0.05). Expression levels of neurexin and neuroligin in ENS are significantly down-regulated in HSCR, which may be involved in the pathogenesis of HSCR.
为了研究神经连接蛋白和神经黏连蛋白在先天性巨结肠症（HSCR）肠神经系统（ENS）中的表达水平。通过对新鲜的肠道壁进行解剖，从小鼠、豚鼠和人类中获得带有附着肠系膜丛的纵向肌肉。通过免疫荧光染色对神经连接蛋白 I 和 Hu（神经元标志物）、神经黏连蛋白 1 和 Hu、神经连接蛋白 I 和突触小泡蛋白（突触前标志物）以及神经黏连蛋白 1 和 PSD95（突触后标志物）进行双重标记。将图像合并以确定蛋白质的相对定位。通过免疫组织化学研究 HSCR 中 ENS 不同节段的神经连接蛋白和神经黏连蛋白的表达水平。在 HSCR 小鼠、豚鼠和人类的肠系膜丛中检测到神经连接蛋白和神经黏连蛋白。神经连接蛋白位于突触前，而神经黏连蛋白位于突触后。神经连接蛋白和神经黏连蛋白在 HSCR 的结肠节段表达水平显著，在过渡段中度，在无神经节结肠段阴性。与正常节段相比，过渡段神经连接蛋白和神经黏连蛋白的表达水平显著下调（P<0.05）。HSCR 中 ENS 的神经连接蛋白和神经黏连蛋白表达水平显著下调，这可能与 HSCR 的发病机制有关。
