唐氏综合征动物模型肠神经系发育的动态观察

The development of the enteric nervous system in terisomy 16 mice with the occurrence of congenital megacolon

目的通过研究唐氏综合征动物模型１６三体鼠肠神经系发育，揭示１６三体鼠是否伴有先天性巨结肠。方法通过免疫组织化学方法测定１６三体胎鼠及同龄胎鼠蛋白基因产物９．５（ＰＧＰ９．５），并观察了肠神经系的形态学特征。结果１６三体胎鼠肠神经系发育异常，肠肌间神经丛发育迟缓，肠粘膜下神经丛缺如，盲肠末端有５ｍｍ的无神经节区。但肠道外源性神经支配正常，肠系膜神经系发育良好。结论唐氏综合征动物模型１６三体鼠伴有先天性巨结肠，这种动物模型可用于研究先天性巨结肠病。

Objectives To study the developmental defect of the enteric nervous system in trisomy 16 mice, an animal model for Down syndrome, and reveal the possibility of trisomy 16 mice with congenital megacolon. Methods We performed trisomy 16 mouse breeding, cytogenetic analysis of trisomy 16 mouse embryos and their normal litteermates from embryo day 13 18 (ED13 ED18), protein gene product 9.5 (PGP 9.5)immunohistochemistry in section and whole mount preparations. Results Developmental delay of ENS was present in trisomy 16 mouse embryos by comparison with their normal littermates. No submucosal plexus was found in gut except 5 mm aganglionic bowel aparting from anus in trisomy 16 mice. The developed mesentery nervous fibers were shown as well as the innervated gut of trisomy 16 mouse embryos and their normal littermates. Conclusion Trisomy 16 mice occur with congenital megacolon, and trisomy 16 mice may be also regard as an animal model for Hirschsprung's disease.