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Nitric oxide and endothelin in pathophysiology of Hirschsprung's disease

一氧化氮和内皮素在先天性巨结肠病病理生理学中的作用

基本信息

批准号：
09671841
负责人：
HAMADA Yoshinori
金额：
$ 1.92万
依托单位：
Kansai Medical University
依托单位国家：
日本
项目类别：
Grant-in-Aid for Scientific Research (C)
财政年份：
1997
资助国家：
日本
起止时间：
1997 至 1999
项目状态：
已结题

来源：
https://kaken.nii.ac.jp/grant/KAKENHI-PROJECT-09671841/
关键词：
Nitric oxide Endothelin Hirschsprung's disease EDNRB gene

项目摘要

Immunohistochemistry of bNOS, eNOS, and iNOS was performed on resected specimen of Hirschsprung's disease. Localization of eNOS and iNOS was observed in vascular endothelium and in submucosal layer to mucosa, respectively in the control specimen, which were obtained from colostomy in patients with anorectal malformation. In the Hirschsprung's disease, no significant differences were observed between aganglionic and normoganglionic segment, and findings of localization were as same as those in control bowel. NADPH immunohistochemistry also showed nearly same results as reported before. However, bNOS localization was found in the nerve bundles appeared in the intermuscular layer of the aganglionic segment.Immunohistochemistry endothelin 3 and endothelin 1 receptor was also performed. In the control bowel, endothelin 3 and endothelin 1 receptor were strongly localized in the submucosal plexus and mucosal muscle layer and also in the both muscle layers and intermuscular plexus. Affected bowel of Hirschsprung's disease did not show any differences in immunohistochemical findings as compared with those in controls.Autoradiography of endothelin A receptor (EDNRA) and endothelin B receptor (EDNRB) was performed using freshly frozen section of resected bowels. In the control bowel, EDNRA was localized weakly in the submucosal layer and EDNRB was found not only in the proper muscle layer but also in both the intermuscular and submucosal plexus. In the affected segment of Hirschsprung's disease, EDNRB was localized in the nerve bundles in the intermuscular layer.Mutational analysis of EDNRB gene was performed by PCR-SSCP method using peripheral leucocytes from Hirschsprung's disease. No mutation was found in all seven exons of the EDNRB gene even in a patient with congenital central hypoventilation syndrome.

对切除的巨结肠标本进行bNOS、eNOS和iNOS的免疫组化。在肛肠畸形患者结肠造口获得的对照标本中，分别观察到eNOS和iNOS在血管内皮和粘膜下至粘膜层的定位。在Hirschsprung病中，在神经节节段和正神经节节段之间没有观察到显著差异，定位结果与对照肠相同。NADPH免疫组化也显示与之前报道的结果几乎相同。然而，bNOS定位于神经节段肌间层的神经束。免疫组化内皮素3和内皮素1受体。在对照肠中，内皮素3和内皮素1受体强烈定位于粘膜下神经丛和粘膜肌层，也存在于肌层和肌间神经丛中。与对照组相比，患先天性巨结肠病的肠道在免疫组化结果上没有任何差异。采用新鲜冷冻的切除肠壁切片进行内皮素A受体（EDNRA）和内皮素B受体（EDNRB）放射自显影。在对照肠中，EDNRA在粘膜下层中定位较弱，EDNRB不仅在固有肌肉层中发现，而且在肌间丛和粘膜下丛中也发现。在Hirschsprung病的受累节段，EDNRB定位于肌间层的神经束。利用巨结肠病外周血白细胞，采用PCR-SSCP方法对EDNRB基因进行突变分析。即使在患有先天性中枢性低通气综合征的患者中，EDNRB基因的所有七个外显子也未发现突变。

项目成果

期刊论文数量（0）

专著数量（0）

科研奖励数量（0）

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专利数量（0）

高田晃平, 浜田吉則, 松岡美子, 棚野晃秀, 加藤泰規, 佐藤正人, 真田俊明, 辻正純, 古形宗久, 奥田和之, 高橋伯夫, 日置紘士郎: "ヒルシュスプルング病におけるエンドセリン受容体に関する免疫組織的検討"日本小児外科学会雑誌. 33(3). 492 (1997)

Kohei Takada、Yoshinori Hamada、Yoshiko Matsuoka、Akihide Tanano、Yasunori Kato、Masato Sato、Toshiaki Sanada、Masazumi Tsuji、Munehisa Furugata、Kazuyuki Okuda、Hakuo Takahashi、Koshiro Hioki：“关于先天性巨结肠症中的内皮素受体”免疫组织学研究”杂志日本小儿外科学会。33（3）492（1997）。