Trisomy 16鼠结肠和人先天性巨结肠蛋白基因产物9.5的表达

目的：研究Ｄｏｗｎ’ｓ 综合征动物模型ｔｒｉｓｏｍｙ １６ 结肠神经系发育和先天性巨结肠（ＨＤ） 病变肠管蛋白基因产物９－５（ｐｒｏｔｅｉｎ ｇｅｎｅ ｐｒｏｄｕｃｔ９ ．５ ，ＰＧＰ９－５） 的神经表达。方法：Ｔｒｉｓｏｍｙ １６ 鼠培育；细胞遗传学分析；Ｔｒｉｓｏｍｙ １６ 鼠结肠和ＨＤＰＧＰ９－５ 免疫组织化学。结果：（１）Ｔｒｉｓｏｍｙ １６ 鼠结肠神经系发育异常，肌间神经丛发育迟缓，粘膜下神经丛缺失，结肠末端有５ ｍｍ 的无神经节区，但结肠系膜神经发育良好；（２）ＨＤ狭窄段肠管ＰＧＰ９－５ 阳性神经纤维大量增生，神经节细胞缺如。结论：（１）Ｔｒｉｓｏｍｙ １６ 鼠具有稳定的遗传学特征，可能伴先天性巨结肠。（２） 由于ＨＤ 狭窄段肠管神经节细胞缺失，增生的ＰＧＰ９－５ 阳性神经纤维是肠道外源性神经的代偿，对其神经元的性质尚有待确定。（３）ＨＤ有遗传倾向

The expression of protein gene product 9.5 in trisomy 16 mouse colon and Hirschsprung' s disease

AIM:To study the developmental defect of colon enteric nervous system in trisomy 16 mice, an animal modal for Down's syndrome, and expression of protein gene product 9.5 (PGP 9.5) in the stenosed segment of Hirschsprung's disease (HD).METHODS:Trisomy 16 mouse breeding; cytogenital of trisomy 16 mouse embryos and their normal littermates and PGP 9.5 immunohistochemistry of trisomy 16 mice and HD. RESULTS:(A) Developmental delay of enteric nervous system was present in trisomy 16 mouse colon. There were no submucosal plexus in the colon and 5 mm aganglionic bowel in length aparting from anus in trisomy 16 mice. However, the developed mesentery nervous fibers were shown to innervate the aganglionic segment. (B) A great proliferation of PGP 9.5-positive nervous fibers was found in the stenosed segment of HD, but there was no neurons.CONCLUSIONS:(A) Trisomy 16 mice may also regard as an animal model of Hirschsprung's disease for their stabilize hereditary feature. (B) Those PGP 9.5-positive fibers may be the compensation of exotic nervous fibers because of no ganglionic cells in the stenosed segment of HD. (C) HD has genetic tendency.