Duchenne muscular dystrophy (DMD) is characterized by a pronounced and progressive degradation of the structure of skeletal muscles, which decreases their strength and lowers endurance of the organism. At muscular dystrophy, mitochondria are known to undergo significant functional changes, which is manifested in a decreased efficiency of oxidative phosphorylation and impaired energy metabolism of the cell. It is believed that the DMD-induced functional changes of mitochondria are mainly associated with the dysregulation of Ca2+ homeostasis. This work examines the kinetic parameters of Ca2+ transport and the opening of the Ca2+-dependent MPT pore in the skeletal-muscle mitochondria of the dystrophin-deficient C57BL/10ScSn-mdx mice. As compared to the organelles of wild-type animals, skeletal-muscle mitochondria of mdx mice have been found to be much less efficient in respect to Ca2+ uniport, with the kinetics of Na+-dependent Ca2+ efflux not changing. The data obtained indicate that the decreased rate of Ca2+ uniport in the mitochondria of mdx mice may be associated with the increased level of the dominant negative subunit of Ca2+ uniporter (MCUb). The experiments have also shown that in mdx mice, skeletal-muscle mitochondria have low resistance to the induction of MPT, which may be related to a significantly increased expression of adenylate translocator (ANT2), a possible structural element of the MPT pore. The paper discusses how changes in the expression of calcium uniporter and putative components of the MPT pore caused by the development of DMD can affect Ca2+ homeostasis of skeletal-muscle mitochondria.

中文翻译：

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杜氏肌营养不良症与线粒体中钙单向的抑制和细胞器对钙诱导的通透性转变的敏感性增加有关。

杜兴氏肌营养不良症（DMD）的特征是骨骼肌结构的明显进行性退化，这会降低其强度并降低生物体的耐力。在肌营养不良症中，线粒体会发生显着的功能变化，这表现为氧化磷酸化效率降低和细胞能量代谢受损。可以相信，DMD诱导的线粒体功能改变主要与Ca2 +稳态失调有关。这项工作检查了肌钙蛋白缺乏的C57BL / 10ScSn-mdx小鼠骨骼肌线粒体中Ca2 +转运的动力学参数和Ca2 +依赖性MPT孔的开放。与野生动物的细胞器相比 已经发现，mdx小鼠的骨骼肌线粒体相对于Ca2 +单端口效率低得多，并且依赖Na +的Ca2 +外排动力学没有改变。获得的数据表明，mdx小鼠线粒体中Ca2 +单端口减少的速率可能与Ca2 +单端口（MCUb）的显性负亚基水平升高有关。实验还表明，在mdx小鼠中，骨骼肌线粒体对MPT的诱导具有较低的抵抗力，这可能与MPT孔的可能结构元件腺苷酸转运蛋白（ANT2）的表达显着增加有关。本文讨论了DMD的发展引起的钙单向转运蛋白表达和MPT孔假定成分的变化如何影响骨骼肌线粒体的Ca2 +稳态。