School of Biological Sciences, University of Reading, Reading, UK.
College of Medicine, Wasit University, Kut, Iraq.
Sci Rep. 2019 Feb 26;9(1):2770. doi: 10.1038/s41598-019-39532-4.
The dystrophin-glycoprotein complex (DGC) links the muscle cytoskeleton to the extracellular matrix and is responsible for force transduction and protects the muscle fibres from contraction induced damage. Mutations in components of the DGC are responsible for muscular dystrophies and congenital myopathies. Expression of DGC components have been shown to be altered in many myopathies. In contrast we have very little evidence of whether adaptive changes in muscle impact on DGC expression. In this study we investigated connection between muscle fibre phenotype and the DGC. Our study reveals that the levels of DGC proteins at the sarcolemma differ in highly glycolytic muscle compared to wild-type and that these changes can be normalised by the super-imposition of an oxidative metabolic programme. Importantly we show that the metabolic properties of the muscle do not impact on the total amount of DGC components at the protein level. Our work shows that the metabolic property of a muscle fibre is a key factor in regulating the expression of DGC proteins at the sarcolemma.
肌营养不良蛋白聚糖复合物（DGC）将肌肉细胞骨架与细胞外基质连接起来，负责力的转导，并保护肌肉纤维免受收缩引起的损伤。DGC 成分的突变导致肌肉营养不良症和先天性肌病。在许多肌病中，已经表明 DGC 成分的表达发生了改变。相比之下，我们几乎没有证据表明肌肉的适应性变化是否会影响 DGC 的表达。在这项研究中，我们研究了肌纤维表型与 DGC 之间的联系。我们的研究表明，与野生型相比，高度糖酵解肌肉的肌细胞膜上 DGC 蛋白的水平不同，而通过叠加氧化代谢方案可以使这些变化正常化。重要的是，我们表明肌肉的代谢特性不会影响蛋白水平上 DGC 成分的总量。我们的工作表明，肌纤维的代谢特性是调节肌细胞膜上 DGC 蛋白表达的关键因素。
