先天性巨结肠Bcl-2的表达及意义

1886年丹麦医生Hirschsprung首先描述了先天性巨结肠症,故又称为Hirschsprung's Disease(HD)。HD发病率居先天性畸形的第二位,平均每5000个成活新生儿中发生1例,其病理学基础是病变肠段结肠壁神经节细胞缺如。但HD的病因较为复杂,迄今仍有争议。Bcl-2蛋白是凋亡抑制基因Bcl-2的表达产物,具有遏止细胞凋亡的作用。在正常神经系统发育过程中,Bcl-2蛋白在外周神经系统中长期表达,提示Bcl-2蛋白对外周神经系统神经元的存活起了重要的作用,尤其是外周神经受到伤害时,Bcl-2蛋白有助于外周神经的修复。目前,Bcl-2蛋白对神经

Bcl-2 EXPRESSION IN ENTERIC NEURONS OF HIRSCHSPRUNG'S DISEASE AND ITS SIGNIFICANCE

The bcl-2 protein, which widely expressed in the developing central and peripheral nervous systems, has the functional role of blocking apoptosis. The purpose of this study was to map bcl-2 expression in the human enteric nervous system and investigate the value of bcl-2 immunohistochemical method in the diagnosis of Hirsehsprung's disease (HD), as this has not previously been done. Rectal specimens were obtained from definitive operation of 20 patients with HD. Specimens were analyzed with immunohistochemical methods, using antibodies against bcl-2. The bcl-2 protein was expressed in myenteric and submucous neurons in normal adult and HD expand segment,but no bcl-2 immunoreactive enteric neurons was revealed in the narrow segment. And nerve fibers of the enteric plexuses that were bcl-2 immunoreactive were few in all examined specimens.From the conclusion,expression of bcl-2 is displayed in enteric neurons and immunohistochemical analysis of bcl-2 may also be valuable for identification of the enteric neurons in HD.