Fabbrizio E, Leger J, Leger J J, Mornet D
INSERM U 300, Physiopathologie expérimentale, Faculté de Pharmacie, Montpellier, France.
Neurosci Lett. 1993 May 28;155(1):51-6. doi: 10.1016/0304-3940(93)90671-7.
The presence of different dystrophin-related protein forms was investigated in electric organ as compared to cardiac, white or red skeletal muscles from Torpedo marmorata. Two strategies were followed. First, we used specific C-terminal dystrophin and dystrophin-related protein monoclonal antibodies which we characterized in the present study. 400 kDa protein bands were detected in the tissues mentioned above with both specific types of antibodies. Second, we produced monoclonal antibodies raised against a dystrophin-enriched preparation from T. marmorata electric organ. Western blot and immunofluorescence analyses showed the tissue specificity of T. marmorata antibodies and allowed us to classify them as types I, II and III. Vessel walls and neuromuscular junctions were labeled with T. marmorata type II and III antibodies in human muscles (skeletal and smooth). Both approaches demonstrated that the T. marmorata electric organ contained different proteins related with dystrophin: a dystrophin form, a dystrophin-related protein form and a dystrophin-related protein isoform, homologous to the dystrophin-related protein present in muscle vessel walls and at the neuromuscular junctions of human tissues. The presence of dystrophin and dystrophin-related protein is finally discussed relative to their functions and organ specificities.
与斑纹电鳐的心脏、白色或红色骨骼肌相比，研究了电鳐电器官中不同抗肌萎缩蛋白相关蛋白形式的存在情况。采用了两种策略。首先，我们使用了特异性的抗肌萎缩蛋白C端和抗肌萎缩蛋白相关蛋白单克隆抗体，这些抗体在本研究中进行了表征。用这两种特异性抗体在上述组织中均检测到了400 kDa的蛋白条带。其次，我们制备了针对斑纹电鳐电器官中富含抗肌萎缩蛋白的制剂的单克隆抗体。蛋白质免疫印迹和免疫荧光分析显示了斑纹电鳐抗体的组织特异性，并使我们能够将它们分为I型、II型和III型。在人体肌肉（骨骼肌和平滑肌）中，斑纹电鳐II型和III型抗体标记了血管壁和神经肌肉接头。两种方法均表明，斑纹电鳐电器官含有与抗肌萎缩蛋白相关的不同蛋白质：一种抗肌萎缩蛋白形式、一种抗肌萎缩蛋白相关蛋白形式和一种抗肌萎缩蛋白相关蛋白异构体，它们与人体组织血管壁和神经肌肉接头中存在的抗肌萎缩蛋白相关蛋白同源。最后讨论了抗肌萎缩蛋白和抗肌萎缩蛋白相关蛋白的存在与其功能和器官特异性的关系。
