Chevron M P, Demaille J
CRBM-CNRS-INSERM U249, Montpellier, France.
C R Seances Soc Biol Fil. 1994;188(5-6):575-84.
Dystrophin, the Duchenne muscular Dystrophy gene product, is a large cytoskeletal protein associated with a complex of membrane proteins, the Dystrophin Glycoprotein Complex (DGC). Dystrophin is localized to the sarcolemmal membrane of all normal muscle types, but is absent from muscles of DMD patients. Using monoclonal antibodies raised against distinct regions of the dystrophin, we studied its expression and subcellular localization during human skeletal, cardiac and smooth muscle development. We have shown that the expression and the association of dystrophin with the plasma membrane take place earlier in cardiac and smooth muscles (8 weeks of gestation) than in skeletal muscles. In skeletal muscles, dystrophin is first observed in the cytoplasm, and is progressively localized to the plasma membrane from 10 weeks onwards. We obtained differences in staining when using antibodies against either the central part of the protein or the carboxy-terminal domain, and we suggested that isoforms of dystrophin, probably differing in their carboxy-terminal end and their capacity to associate with the plasma membrane were differentially expressed during development and in different tissue-types (7). These findings are discussed in the context of the pathology of Duchenne Muscular Dystrophy.
肌营养不良蛋白是杜兴氏肌营养不良症的基因产物，是一种大型细胞骨架蛋白，与膜蛋白复合物——肌营养不良蛋白糖蛋白复合物（DGC）相关联。肌营养不良蛋白定位于所有正常肌肉类型的肌膜，但在杜兴氏肌营养不良症患者的肌肉中不存在。我们使用针对肌营养不良蛋白不同区域产生的单克隆抗体，研究了其在人类骨骼肌、心肌和平滑肌发育过程中的表达及亚细胞定位。我们已经表明，肌营养不良蛋白的表达及其与质膜的结合在心肌和平滑肌（妊娠8周）中比在骨骼肌中发生得更早。在骨骼肌中，肌营养不良蛋白首先在细胞质中被观察到，并从10周起逐渐定位于质膜。当使用针对该蛋白中央部分或羧基末端结构域的抗体时，我们获得了不同的染色结果，并且我们认为，肌营养不良蛋白的亚型可能在其羧基末端以及与质膜结合的能力方面存在差异，在发育过程中和不同组织类型中差异表达（7）。这些发现将在杜兴氏肌营养不良症的病理学背景下进行讨论。
