Impaired expression of neural cell adhesion molecule L1 in the extrinsic nerve fibers in Hirschsprung's disease
Impaired expression of neural cell adhesion molecule L1 in the extrinsic nerve fibers in Hirschsprung's disease
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DOI：
10.1016/s0022-3468(97)90703-x
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发表时间：
1997-04-01
影响因子：
2.4
通讯作者：
Kawamura, K
中科院分区：
文献类型：
--
作者：
Ikawa, H;Kawano, H;Kawamura, K
使用抗三种神经膜蛋白 Thy-1、整合素 α 5 和 ii 的抗体对先天性巨结肠症 (HD) 中的神经节段和无神经节段进行免疫组织化学研究。用针对神经丝（神经元细胞骨架蛋白）的抗体对肠神经元件进行免疫染色。在神经节段中，在神经元细胞体以及肌间神经丛和粘膜下神经丛的细神经纤维中检测到神经丝免疫反应性。所有这些神经元件的 Thy-1、整合素 α 5 和 ii 均呈免疫阳性。在无神经节段中，没有检测到内在神经元，而是通过神经丝免疫化学在肌间隙、粘膜下层和环肌层中观察到肥大的神经束。这些肥大的神经束抗 Thy-1 和抗整合素 α 5 抗体呈免疫阳性。然而，在所有 5 例病例中，它们都没有被抗 ii 免疫染色。这些发现表明，在细胞粘附、神经细胞迁移和神经突生长中起重要作用的ii分子的表达在无神经节结肠的外在神经纤维中受损，这可能会扰乱神经嵴迁移和充分的神经突生长，从而导致HD中的无神经节段和外在纤维的异常神经束。版权所有 (C) 1997 W.B.桑德斯公司。
Immunohistochemical studies on the ganglionic and aganglionic segment in Hirschsprung's disease (HD) were carried out using antibodies against three neural membrane proteins, Thy-1, Integrin alpha 5, and ii. Enteric neural elements were immunostained with antibodies against neurofilament, which is the neuronal cytoskeletal protein. In ganglionic segments, neurofilament-immunoreactivity was detected in neuronal cell bodies and fine nerve fibers of the myenteric and submucosal plexuses. All of these neural elements were immunopositive for Thy-1, integrin alpha 5, and ii. In aganglionic segments, no intrinsic neurons were detected, and instead, hypertrophied nerve bundles were observed in intermuscular space, in submucosa, and in circular muscle layer by immunochemistry for neurofilament. These hypertrophied nerve bundles were immunopositive with anti-Thy-1 and anti-integrin alpha 5 antibodies. However, they were not immunostained with anti-ii in all five cases. These findings indicate that the expression of ii molecule, which plays an important role in cell adhesion, neural cell migration, and neurite outgrowth, is impaired in the extrinsic nerve fibers in aganglionic colon, And this may perturb neural crest migration and adequate neurite outgrowth, with resulting aganglionic segment and abnormal nerve bundles of extrinsic fibers in HD. Copyright (C) 1997 by W.B. Saunders Company.