CAJ | 학술논문

目的研究骨形成蛋白Ⅱ型受体(bone morphogenetic protein receptor Ⅱ,BMPR Ⅱ)在局灶性大脑皮质发育不良(focal cortical dysplasia,FCD)中的表达.方法收集自2008年6月至2010年6月手术切除并经病理检测证实的皮质发育障碍(FCDⅡb型)标本14例,通过免疫组化、免疫荧光双标以及免疫印迹方法,检测BMPRⅡ在正常脑组织与病理标本中的表达分布.结果在正常脑组织中,BMPRⅡ广泛表达于灰质神经元中,在白质区未见表达.在FCDⅡb病理标本中,BMPRⅡ强表达于FCDⅡb特征性的气球样细胞(balloon cells,BCs)、发育不良神经元(dysplastic neurons,DNs)以及巨大神经元(giant neurons,GNs),此外,在反应性星形胶质细胞上也有表达,而弱表达于形态正常的神经元(normal-appearing neurons).免疫印迹结果显示,BMPR Ⅱ在FCDⅡb病理标本中表达较正常脑组织减低(P<0.05).结论BMPRⅡ蛋白异常表达于FCDⅡ b致痫灶中,可能参与了皮质发育障碍的发生机制.
목적 연구골형성단백Ⅱ형수체(bone morphogenetic protein receptor Ⅱ,BMPR Ⅱ)재국조성대뇌피질발육불량(focal cortical dysplasia,FCD)중적표체.방법수집자2008년6월지2010년6월수술절제병경병리검측증실적피질발육장애(FCDⅡb형)표본14례,통과면역조화、면역형광쌍표이급면역인적방법,검측BMPRⅡ재정상뇌조직여병리표본중적표체분포.결과 재정상뇌조직중,BMPRⅡ엄범표체우회질신경원중,재백질구미견표체.재FCDⅡb병리표본중,BMPRⅡ강표체우FCDⅡb특정성적기구양세포(balloon cells,BCs)、발육불량신경원(dysplastic neurons,DNs)이급거대신경원(giant neurons,GNs),차외,재반응성성형효질세포상야유표체,이약표체우형태정상적신경원(normal-appearing neurons).면역인적결과현시,BMPR Ⅱ재FCDⅡb병리표본중표체교정상뇌조직감저(P<0.05).결론BMPRⅡ단백이상표체우FCDⅡ b치간조중,가능삼여료피질발육장애적발생궤제.
Objective To detect the expression of bone morphogenetic protein receptor Ⅱ ( BMPR Ⅱ ) in human focal cortical dysplasia ( FCD Ⅱ b). Methods Fourteen specimens of FCD Ⅱ b surgically removed and pathologically verified were collected from June 2008 to June 2010 and the expression of BMPR Ⅱ in the normal brain tissues and the pathological specimens was detected by means of immunohistochemistry and western blot. Results In the normal brain tissues, BMPR Ⅱ was widely expressed in the cortical neurons of the grey matter, with no positive immunostaining in the white matter. In the cortical lesion of FCD Ⅱ b, BMPR Ⅱ was strongly expressed in the misshapen cells including balloon cells (BCs) , dysmorphic neurons (DNs) and giant neurons (GNs). Positive BMPR Ⅱ expression was also observed in the reactive astroeytes and low level expression of BMPR Ⅱ was found in the normal-appearing (NA) neurons. Western-blot analysis showed that BMPR Ⅱ expression tended to be lowered in the FCD Ⅱ b specimens compared with the normal brain tissues ( P < 0. 05 ). Conclusion The expression of BMPR Ⅱ is altered and reduced in the FCD Ⅱ b, suggesting that BMP signal pathway may participate in the pathogenesis of FCD.