Department of Pediatric Surgery, Qilu Hospital, Shandong University, Jinan, Shandong Province, China.
Neurogastroenterol Motil. 2017 Dec;29(12). doi: 10.1111/nmo.13134. Epub 2017 Jun 28.
The goal of this study was to investigate the expression of fibronectin (FN) and the correlated abundance of neuroligins (NLs) in the enteric nervous system (ENS) and to find a novel diagnostic marker in the serum of Hirschsprung disease (HSCR) patients.
The expression levels of FN, neuroligin-1 and neuroligin-2 were detected in 114 children with or without HSCR. The expression and localization of the NLs and FN were assessed morphologically by immunohistochemical staining. Western blot analysis and real-time fluorescence quantitative PCR (qPCR) were performed to examine the correlated expression of the NLs and FN in aganglionic, transitional, and normal ganglionic colon tissues. An enzyme-linked immunosorbent assay (ELISA) was performed to evaluate and compare serum FN levels between HSCR and non-HSCRand between long-type HSCR and short-type HSCR.
These studies showed that both neuroligin-1 and neuroligin-2 were expressed at low levels in aganglionic segments and at intermediate levels in transitional segments compared to their high level of expression in normal tissue. In contrast, FN expression was negatively correlated, with expression in these three samples transitioning from highest to lowest. The serum FN level was higher in HSCR than in non-HSCR, but no significant difference between short-type HSCR and long-type HSCR was observed.
FN affects the expression of both neuroligin-1 and neuroligin-2 in HSCR, which may lead to the hypoplasia of ganglion cells in the ENS. This correlation may play a key role in the pathogenesis, diagnosis, or classification of HSCR.
本研究旨在探讨纤维连接蛋白（FN）在肠神经（ENS）中的表达及其与神经连接蛋白（NLs）的相关性，并寻找先天性巨结肠（HSCR）患者血清中的一种新的诊断标志物。
检测了 114 例伴有或不伴有 HSCR 儿童的 FN、神经连接蛋白-1 和神经连接蛋白-2 的表达水平。通过免疫组织化学染色评估 NLs 和 FN 的表达和定位。Western blot 分析和实时荧光定量 PCR（qPCR）检测无神经节细胞、过渡和正常神经节细胞结肠组织中 NLs 和 FN 的相关性表达。通过酶联免疫吸附试验（ELISA）评估和比较 HSCR 和非 HSCR 以及长段 HSCR 和短段 HSCR 患者血清 FN 水平。
这些研究表明，神经连接蛋白-1 和神经连接蛋白-2 在无神经节细胞段的表达水平较低，在过渡段的表达水平中等，而在正常组织中的表达水平较高。相比之下，FN 的表达呈负相关，这三种样本的表达水平从高到低依次降低。HSCR 患者的血清 FN 水平高于非 HSCR 患者，但短段 HSCR 和长段 HSCR 之间无显著差异。
FN 影响 HSCR 中神经连接蛋白-1 和神经连接蛋白-2 的表达，可能导致 ENS 神经节细胞发育不良。这种相关性可能在 HSCR 的发病机制、诊断或分类中起关键作用。
