Increased population of immature enteric glial cells in the resected proximal ganglionic bowel of Hirschsprung's disease patients
Increased population of immature enteric glial cells in the resected proximal ganglionic bowel of Hirschsprung's disease patients
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DOI：
10.1016/j.jss.2017.05.062
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发表时间：
2017-10-01
影响因子：
2.2
通讯作者：
Puri, Prem
中科院分区：
文献类型：
--
作者：
Tani, Gakuto;Tomuschat, Christian;Puri, Prem
背景：肠神经胶质细胞是正常胃肠功能所必需的。神经胶质结构、发育或功能的异常会导致胃肠道生理学的紊乱。脂肪酸结合蛋白7(FABP7)是未成熟的肠神经胶质细胞的标志，而S100只在成熟的神经胶质细胞中表达。先天性巨结肠症(HSCR)患者即使进行了适当的手术，也经常会出现运动障碍和小肠结肠炎。方法：研究FABP7、S100和PGP 9.5在HSCR患者(n=6)和正常对照组(n=6)神经节和无神经节段肠组织中的表达。用免疫荧光和共聚焦显微镜检测蛋白质分布。结果：定量聚合酶链式反应和免疫印迹分析显示FABP7在神经节细胞中的表达显著高于对照组(P<0.05)。共聚焦显微镜下可见FABP7(+)胶质细胞位于结肠上皮下，并与节段性肠段的肠神经元紧密相连。结论：HSCR患者节段性肠段内未成熟的肠胶质细胞(EGCs)数量显著增加，可能对肠神经元功能和肠屏障功能产生不利影响，从而使HSCR患者易患肠动力障碍和肠结肠炎。(C)2017 Elsevier Inc.保留所有权利。
Background: Enteric glial cells are essential for normal gastrointestinal function. Abnormalities in glial structure, development, or function lead to disturbances in gastrointestinal physiology. Fatty acidebinding protein 7 (FABP7) is a marker of immature enteric glial cells, whereas S100 is expressed only by mature glial cells. Patients with Hirschsprung's disease (HSCR) often suffer from dysmotility and enterocolitis despite proper surgery. We designed this study to determine the distribution and expression of glial cells in patients with HSCR compared to normal controls.Methods: We investigated FABP7, S100, and PGP 9.5 expressions in both the ganglionic and aganglionic bowel of patients with HSCR (n = 6) versus normal control colon (n = 6). Protein distribution was assessed by using immunofluorescence and confocal microscopy. Gene and protein expressions were quantified using quantitative real-time polymerase chain reaction (qPCR), Western blot analysis, and densitometry.Results: qPCR and Western blot analysis demonstrated a significantly increased FABP7 expression in ganglionic specimens compared to control specimen (P < 0.05). Confocal microscopy revealed FABP7(+) glia cells lie under the colonic epithelium and in close apposition to enteric neurons in the ganglionic bowel.Conclusions: The significantly increased number of immature enteric glial cells (EGCs) in the ganglionic bowel of HSCR patients may have adverse effect on the function of enteric neurons and intestinal barrier and thus predispose these patients to intestinal motility problems and enterocolitis. (C) 2017 Elsevier Inc. All rights reserved.