摘要
目的探讨免疫荧光检测对Duchenne型、Becker型和肢带型肌营养不良症(DMD、BMD和LG-MD)临床诊断的价值。方法对25例肌营养不良症患者(DMD 10例,BMD 4例,LGMD 11例)的骨骼肌冰冻切片标本应用免疫荧光法检测抗肌萎缩蛋白(Dys)中央棒状区(Dys1)、C-末端(Dys2)、N-末端(Dys3)单克隆抗体及α、-β、-γ-肌聚糖蛋白(SG)多克隆抗体在肌膜的表达。结果10例DMD患者Dys染色均为阴性,4例BMD患者呈弱阳性;11例LGMD患者的SG染色中,分别有1例α-SG阴性及1例β-SG阴性。结论Dys免疫荧光检测对DMD/BMD的临床诊断具有特异性价值,是临床诊断的可靠方法之一;SG检测对LGMD的临床诊断还需进一步完善。
Objective To explore the value of immunofluorescent technique for clinical diagnosing Duchenne muscular dystrophy （ DMD ）, Becker muscular dystrophy （ BMD ） and Limb-girdle muscular dystrophy （ LGMD ）. Methods Immunofluorescent technique was applied, and the expressions of Dysl, Dys2, Dys3 monoclonal antibodies and α-, β-, γ-sarcoglycan （SG） polyclonal antibodies against dystrophin, α-SG, β-SG, γ-SG in musculomembranes of frozen section specimens from 25 patients （ 10 cases of DMD, 4 cases of BMD and 11 cases of LGMD） were detected. Results 10 DMD patients had negative staining of dystrophin, and 4 BMD patients had discontinuous or a patchy positive staining pattern. All LGMD patients had positive dystrophin staining. There was one patient presented negative staining of α-SG and β-SG, respectively. Conclusions Detecting of dystrophin by immunofluorescent technique is special and helpful in diagnosing and classifying DMD/BMD. At present, SG may not be used in diagnosing the LGMD patients.
出处
《临床神经病学杂志》
CAS
北大核心
2006年第6期403-405,共3页
Journal of Clinical Neurology
基金
安徽省自然科学基金资助项目(01043710)
