摘要
目的探讨α-共核蛋白(-αsynuclein,-αsyn)和神经纤维丝(neurofilaments,NFs)的表达与先天性巨结肠症(Hirschsprung's disease,HD)发病机制之间的关系。方法采用过氧化物酶标记的链霉卵白素法,即SP法免疫组织化学技术,观察-αsyn,NFs在36例HD病人的扩张段、移行段、狭窄段和4例无消化道畸形的尸解结肠标本中的分布情况。结果36例HD病人肠管的扩张段、移行段肌间和粘膜下丛节细胞中有19例-αsyn呈阳性表达,狭窄段神经纤维束增粗,呈阳性染色,节细胞缺如,阳性率为52.8%,36例HD病人肠管的扩张段、移行段肌间和粘膜下丛节细胞都对NFs呈强阳性表达,阳性率100%,4例尸解结肠标本的神经节细胞对-αsyn呈阴性表达,对NFs免疫反应性呈阴性或弱阳性。结论-αsyn和NFs分布异常与HD的发生有密切关系,胚胎期某种致病因素可能不仅影响神经嵴细胞的移行,也影响神经的分化和成熟。
Objective To observe the distribution of α-synuclein and neurofilaments in aganglionic,transitional and dilated segments of colon in Hirschsprung＇s disease,and explore the relationship between the expressions of α-syn and NFs and pathogenesis of HD. Methods The distributions of α-syn and NFs and 4 specimens of normal clone of autopsy were studies using streptaridin-peroxidase,i, e. sp immunohistochemistry approach. Results The positive staining of α-syn in 19 out of 36 ganglion cell of aganglionic,transitional segments of both the myenteric and submu- cosal plexuses,α-syn immunoreactivity was found in hypertrophic nerve fascicules, absence of ganglion cell in 19 cases （52.8 %）. The strong positive staining of NFs in 36 （100%）. The negative staining of α-syn and the negative or weak positive immuoreactivity staining of NFs were shown in ganglion cell of inter：muscular 4 specimens of autopsy. Conclusion Abnormal distribution of α-syn and NFs are closely related to pathogenesis of HD. It＇s suspected that certain factor differentiation and maturation of nerve.
出处
《江西医学院学报》
2005年第5期50-52,共3页
Acta Academiae Medicinae Jiangxi
