Pons F, Nicholson L V, Robert A, Voit T, Leger J J
Institut National de la Santé et de la Recherche Médicale, INSERM U300, Faculté de Pharmacie, Montpellier, France.
Neuromuscul Disord. 1993 Sep-Nov;3(5-6):507-14. doi: 10.1016/0960-8966(93)90106-t.
The respective localizations of dystrophin and dystrophin-related protein (DRP or utrophin) along the sarcolemmal membrane and at the neuromuscular junctions (NMJs) in normal and dystrophin-deficient skeletal muscles, were determined using confocal laser microscopy. The analysis was prompted by the recent availability of a new anti-utrophin mAb [Bewick et al. NeuroReport 1992; 3:857-860] and different mAbs that react with dystrphin or both dystrophin and utrophin. In dystrophin-deficient muscles, utrophin was expressed and detectable over large subcellular areas normally occupied by dystrophin along the sarcolemmal membranes and at the NMJs. Utrophin was expressed in a non-uniform, discontinuous way on the sarcolemmal membrane in dystrophin-deficient skeletal muscles, similar to dystrophin in normal muscle fibres. The respective distributions of both related muscle proteins and their positions relative to the alpha-bungarotoxin acetylcholine (ACh) receptor marker were determined. Double-staining experiments and superimposition of the confocal images showed that utrophin was more closely associated with ACh receptors than dystrophin at the NMJs in normal muscles. Utrophin distribution consequently differed from that of dystrophin.
利用共聚焦激光显微镜确定了肌营养不良蛋白和肌营养不良蛋白相关蛋白（DRP或抗肌萎缩蛋白）在正常和缺乏肌营养不良蛋白的骨骼肌肌膜以及神经肌肉接头（NMJ）处的各自定位。这一分析是由一种新的抗抗肌萎缩蛋白单克隆抗体[Bewick等人，《神经报告》1992年；3：857 - 860]以及与肌营养不良蛋白或肌营养不良蛋白和抗肌萎缩蛋白都反应的不同单克隆抗体的近期可得性所推动的。在缺乏肌营养不良蛋白的肌肉中，抗肌萎缩蛋白在肌膜和神经肌肉接头处通常被肌营养不良蛋白占据的大的亚细胞区域表达且可检测到。在缺乏肌营养不良蛋白的骨骼肌中，抗肌萎缩蛋白以不均匀、不连续的方式在肌膜上表达，类似于正常肌纤维中的肌营养不良蛋白。确定了这两种相关肌肉蛋白的各自分布及其相对于α - 银环蛇毒素乙酰胆碱（ACh）受体标记物的位置。双重染色实验和共聚焦图像叠加显示，在正常肌肉的神经肌肉接头处，抗肌萎缩蛋白比肌营养不良蛋白与ACh受体的关联更紧密。因此，抗肌萎缩蛋白的分布与肌营养不良蛋白不同。
