INRA, UMR 703, Ecole Nationale Vétérinaire de Nantes, Atlanpole - La Chantrerie, BP 40706, Nantes, 44307, France.
Cell Mol Biol Lett. 2010;15(1):134-52. doi: 10.2478/s11658-009-0039-8. Epub 2009 Dec 7.
The dystrophin-glycoprotein complex (DGC) is a large trans-sarcolemmal complex that provides a linkage between the subsarcolemmal cytoskeleton and the extracellular matrix. In skeletal muscle, it consists of the dystroglycan, sarcoglycan and cytoplasmic complexes, with dystrophin forming the core protein. The DGC has been described as being absent or greatly reduced in dystrophin-deficient muscles, and this lack is considered to be involved in the dystrophic phenotype. Such a decrease in the DGC content was observed in dystrophin-deficient muscle from humans with muscular dystrophy and in mice with X-linked muscular dystrophy (mdx mice). These deficits were observed in total muscle homogenates and in partially membrane-purified muscle fractions, the so-called KCl-washed microsomes. Here, we report that most of the proteins of the DGC are actually present at normal levels in the mdx mouse muscle plasma membrane. The proteins are detected in dystrophic animal muscles when the immunoblot assay is performed with crude surface membrane fractions instead of the usually employed KCl-washed microsomes. We propose that these proteins form SDS-insoluble membrane complexes when dystrophin is absent.
肌营养不良蛋白聚糖复合物(DGC)是一个大型跨肌膜复合物,它在肌小节下细胞骨架和细胞外基质之间提供连接。在骨骼肌中,它由肌聚糖、 sarcoglycan 和细胞质复合物组成,肌营养不良蛋白形成核心蛋白。DGC 已被描述为在肌营养不良蛋白缺乏的肌肉中缺失或大大减少,这种缺乏被认为与肌营养不良表型有关。在患有肌营养不良症的人类和患有 X 连锁肌营养不良症(mdx 小鼠)的肌肉中观察到肌营养不良蛋白缺乏的肌肉中 DGC 含量减少。这些缺陷在总肌肉匀浆和部分膜纯化的肌肉部分(所谓的 KCl 洗涤微体)中观察到。在这里,我们报告说,在 mdx 小鼠肌肉质膜中,DGC 的大多数蛋白质实际上以正常水平存在。当使用粗表面膜部分而不是通常使用的 KCl 洗涤微体进行免疫印迹分析时,在营养不良动物肌肉中可以检测到这些蛋白质。我们提出,当肌营养不良蛋白缺失时,这些蛋白质形成 SDS 不溶性膜复合物。