骨骼肌中的肌营养不良蛋白。II. Xp21型肌营养不良患者的免疫反应性。
Dystrophin in skeletal muscle. II. Immunoreactivity in patients with Xp21 muscular dystrophy.
作者信息
Nicholson L V, Davison K, Johnson M A, Slater C R, Young C, Bhattacharya S, Gardner-Medwin D, Harris J B
机构信息
Muscular Dystrophy Group Research Laboratory, Newcastle General Hospital, Newcastle upon Tyne, U.K.
出版信息
J Neurol Sci. 1989 Dec;94(1-3):137-46. doi: 10.1016/0022-510x(89)90224-4.
In the preceding paper a sensitive Western blotting analysis system based on the use of a monoclonal antibody to dystrophin was described. Here we report the immunoreactivity on blots and on unfixed frozen sections of muscle from patients with Duchenne (DMD) and Becker (BMD) muscular dystrophy. Muscle from 3 BMD patients showed variation both in the band pattern observed on blots and in the immunocytochemical labelling of dystrophin on frozen sections. In contrast to previous reports, we were able to detect some minor dystrophin bands on blots from 6 of 9 DMD biopsy samples. Tissue sections from 8 of the 9 contained isolated fibres with dystrophin-positive labelling. We conclude that the majority of DMD patients have muscle fibres which can synthesize dystrophin in a limited manner.
在前一篇论文中，描述了一种基于使用抗肌营养不良蛋白单克隆抗体的灵敏蛋白质印迹分析系统。在此，我们报告了杜兴氏（DMD）和贝克氏（BMD）肌营养不良症患者肌肉的印迹和未固定冰冻切片上的免疫反应性。3例BMD患者的肌肉在印迹上观察到的条带模式以及冰冻切片上肌营养不良蛋白的免疫细胞化学标记方面均表现出差异。与先前的报道相反，我们能够在9例DMD活检样本中的6例的印迹上检测到一些次要的肌营养不良蛋白条带。9例中的8例组织切片含有肌营养不良蛋白阳性标记的孤立纤维。我们得出结论，大多数DMD患者的肌纤维能够以有限的方式合成肌营养不良蛋白。
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