Wiedenmann B, Riedel C, John M, Ahnert-Hilger G, Stoltenburg G, Waldschmidt J, von Deimling K, Riecken E O, Schier F
Virchow-Klinikum Universitätsklinikum Charité, Humboldt University of Berlin, Department of Internal Medicine, Berlin, Germany.
Pediatr Surg Int. 1998 Sep;13(7):468-73. doi: 10.1007/s003830050376.
In Hirschsprung's disease (HD), certain intestinal nervous plexuses are absent. Sprouting nerve endings contain different amounts of synaptophysin (SY), a protein and main constituent of acetylcholinesterase (AChE) storage compartments. Due to the lack of specific markers for synapses, a qualitative analysis of nerve endings of intestinal segments affected by HD has not yet been undertaken. For this study, resected colorectal specimens from patients with HD (n = 8, mean age 2.1 years) were investigated in parallel for AChE, SY, and content of small synaptic vesicles by biochemical, immunohistochemical, and electronmicroscopic means. In the microdissected muscular layer, reduced SY (1.4 microgram/mg total protein, normal 24 +/- 0.3) was observed. Immunohistochemistry showed in affected tissues reduced numbers of SY-positive nerve fibers and nerve endings, which in turn were thickened and distorted, in both the muscle proper and the muscularis mucosae. Combining both morphologic and biochemical findings, in HD the number of cholinergic vesicles in the remaining nerve endings seems to be increased as measured by SY, a marker molecule specific for synaptic vesicles. Our data also suggest that nerve endings in HD may contain high concentrations of cholinergic vesicles, paralleling the known high amounts of acetylcholine and AChE found in intestinal segments of patients with HD.
在先天性巨结肠（HD）中，某些肠神经丛缺失。发芽的神经末梢含有不同量的突触素（SY），这是一种蛋白质，也是乙酰胆碱酯酶（AChE）储存区室的主要成分。由于缺乏突触的特异性标记物，尚未对受HD影响的肠段神经末梢进行定性分析。在本研究中，通过生化、免疫组织化学和电子显微镜方法，对HD患者（n = 8，平均年龄2.1岁）切除的结直肠标本同时进行了AChE、SY和小突触小泡含量的研究。在显微解剖的肌层中，观察到SY减少（1.4微克/毫克总蛋白，正常为24±0.3）。免疫组织化学显示，在受影响的组织中，SY阳性神经纤维和神经末梢数量减少，而在固有肌层和黏膜肌层中，这些神经纤维和神经末梢又增粗且扭曲。综合形态学和生化研究结果，在HD中，通过对突触小泡特异性标记分子SY的检测，剩余神经末梢中胆碱能小泡的数量似乎增加。我们的数据还表明，HD中的神经末梢可能含有高浓度的胆碱能小泡，这与HD患者肠段中已知的高乙酰胆碱和AChE含量相符。
