骨形成蛋白Ⅱ型受体在局灶性大脑皮质发育不良中的表达及作用  

Expression and role of bone morphogenetic protein receptor Ⅱ in focal cortical dysplasia

作　　者：郭为[1] 张春青[1] 余思逊[1] 树海峰[1] 刘仕勇[1] 安宁[1] 杨辉[1] 

机构地区：[1]第三军医大学附属新桥医院神经外科,重庆400037

出　　处：《中华创伤杂志》2011年第5期472-476,共5页Chinese Journal of Trauma

基　　金：国家自然科学基金资助项目（8107104）;国家自然科学基金青年基金资助项目（81000486）;重庆市自然科学基金资助项目（CSTC,2009BB5147）

摘　　要：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.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.

关 键 词：骨形成蛋白受体 Ⅱ型 癫痫 局灶性大脑皮质发育不良 

分 类 号：R742[医药卫生—神经病学与精神病学]