DMD/BMD肌细胞抗肌营养不良蛋白免疫荧光组化研究  

A immunofluorescent study of the dystrophin expressions of muscle cells in patients with DMD/BMD

作　　者：吕佩源[1] 李玲[1] 董艳红[1] 魏慈[1] 崔文柱[1] 赵宝华[1] 王贺波[1] 

机构地区：[1]河北省人民医院神经内科,石家庄050051

出　　处：《脑与神经疾病杂志》2008年第4期320-322,共3页Journal of Brain and Nervous Diseases

基　　金：河北省自然科学基金资助课题(397402)

摘　　要：目的:研究Duchenne/Becker型肌营养不良症(DMD/BMD)患者肌细胞中抗肌营养不良蛋白(dystrophin)的表达及其诊断意义。方法:采用免疫荧光抗体染色技术对5例DMD,2例BMD肌细胞中抗肌营养不良蛋白进行检测,以2例正常人的肌细胞作为以照。结果:对照组肌细胞膜上染色阳性,胞核及胞浆呈阴性;DMD患者肌膜完全无显色;BMD患者染色弱阳性,可见沿肌细胞膜分布的间断斑片状荧光带。结论:抗肌营养不良蛋白缺乏或表达异常是DMD/BMD基本病理基础。应用免疫荧光抗体染色法检测抗肌营养不良蛋白。Objective：To study of dystrophin expressions and the diagnotic significance of muscle cells in patients with Duchenne/Becker muscular dystrophy（DMD/BMD）.Methods：Dystrophin in muscle cells from 5 DMD and 2 BMD were detected by immunofluorescence technique,compared with 2 normal males.Results：In the control group,the normal individuals showed ringed positive staining stripe around muscle cells,the cell nuclei and cytoplasm were negative.Negative result of staining was seen in 5 DMD.The discontinuous pattern positive stain was showed in two BMD cases.Conclusion：The absence or abnormal expression of distrophin was the pathologic foundation of DMD and BMD.Detecting dystrophin in muscles cells by immunofluorescene technique is an efficient method for diagnosising and distinguishing DMD/BMD.

关 键 词：DMD BMD 抗肌营养不良蛋白 免疫荧光技术 

分 类 号：R742[医药卫生—神经病学与精神病学]