Kawajiri M, Mitsui T, Kawai H, Kobunai T, Tsuchihashi T, Saito S
First Department of Internal Medicine, School of Medicine, University of Tokushima, Japan.
J Neuropathol Exp Neurol. 1996 Aug;55(8):896-903. doi: 10.1097/00005072-199608000-00005.
The precise localization and semiquantitative correlation of dystrophin, utrophin and beta-dystroglycan expression on the sarcolemma of skeletal muscle cells obtained from patients with Becker muscular dystrophy (BMD) was studied using three types of double immunofluorescence. Staining intensity was measured using a confocal laser microscope. Each of these proteins was identified at the same locus on the sarcolemma. The staining intensities of dystrophin and utrophin were approximately reciprocal at sarcolemmal sites where dystrophin expression was obviously observed. The staining intensity of beta-dystroglycan was strong in areas where dystrophin staining was also strong and utrophin expression was weak. Quantitative analysis revealed that the staining intensity of beta-dystroglycan minus that of dystrophin approximated the staining intensity of utrophin, indicating that the sum of dystrophin and utrophin expression corresponds to that of beta-dystroglycan. These results suggest that utrophin may compensate for dystrophin deficiency found in BMD by binding to beta-dystroglycan.
利用三种类型的双重免疫荧光技术，研究了从贝克肌营养不良症（BMD）患者获取的骨骼肌细胞肌膜上肌营养不良蛋白、抗肌萎缩蛋白和β-肌营养不良聚糖表达的精确定位和半定量相关性。使用共聚焦激光显微镜测量染色强度。这些蛋白质中的每一种都在肌膜上的同一位置被识别。在明显观察到肌营养不良蛋白表达的肌膜部位，肌营养不良蛋白和抗肌萎缩蛋白的染色强度大致呈反比。在肌营养不良蛋白染色也很强且抗肌萎缩蛋白表达较弱的区域，β-肌营养不良聚糖的染色强度很强。定量分析表明，β-肌营养不良聚糖的染色强度减去肌营养不良蛋白的染色强度近似于抗肌萎缩蛋白的染色强度，这表明肌营养不良蛋白和抗肌萎缩蛋白表达的总和与β-肌营养不良聚糖的表达总和相对应。这些结果表明，抗肌萎缩蛋白可能通过与β-肌营养不良聚糖结合来补偿BMD中发现的肌营养不良蛋白缺乏。
