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Transport of Ca2+ and Ca2+-dependent permeability transition in heart mitochondria in the early stages of Duchenne muscular dystrophy.
Biochimica et Biophysica Acta (BBA) - Bioenergetics ( IF 3.4 ) Pub Date : 2020-06-20 , DOI: 10.1016/j.bbabio.2020.148250
Mikhail V Dubinin 1 , Eugeny Yu Talanov 2 , Kirill S Tenkov 1 , Vlada S Starinets 3 , Irina B Mikheeva 2 , Konstantin N Belosludtsev 3
Affiliation  

Duchenne muscular dystrophy (DMD) is a progressive skeletal muscle disease that is associated with severe cardiac complications in the late stages. Significant mitochondrial dysfunction is reportedly responsible for the development of cardiomyopathy with age. At the same time, adaptive changes in mitochondrial metabolism in cardiomyocytes were identified in the early stages of DMD. In this work, we evaluate the functioning of calcium transport systems (MCU and NCLX), and MPT pore in the heart mitochondria of young dystrophin-deficient mice. As compared to wild-type animals, heart mitochondria of mdx mice have been found to be more efficient both in respect to Ca2+ uniport and Na+-dependent Ca2+ efflux. The data obtained indicate that the increased rate of Ca2+ uptake by heart mitochondria of mdx mice may be due to an increase in the ratio of MCU and MCUb subunits. In turn, an increase in the rate of Ca2+ efflux from organelles in DMD may be the result of a significant increase in the level of NCLX. Moreover, the heart mitochondria of mdx mice were more resistant to MPT pore opening, which may be due to an increase in the microviscosity of mitochondrial membranes of DMD mice. At the same time, the level of putative MPT pore proteins did not change. The paper discusses the effect of rearrangements of the mitochondrial proteome involved in the transport and accumulation of calcium on the adaptation of this organ to DMD.



中文翻译:

在Duchenne肌营养不良的早期,心脏线粒体中Ca2 +和Ca2 +依赖性渗透性转变的转运。

杜氏肌营养不良症(DMD)是一种进行性骨骼肌疾病,在晚期与严重的心脏并发症相关。据报道,随着年龄的增长,线粒体功能障碍是导致心肌病发展的原因。同时,在DMD的早期阶段发现了心肌细胞线粒体代谢的适应性变化。在这项工作中,我们评估了年轻的肌营养不良蛋白缺陷小鼠心脏线粒体中钙转运系统(MCU和NCLX)和MPT孔的功能。与野生型动物相比,发现mdx小鼠的心脏线粒体在Ca 2+单向和依赖Na +的Ca 2+方面都更有效。外排。获得的数据表明,mdx小鼠心脏线粒体摄取Ca 2+的速率增加可能是由于MCU和MCUb亚基比例的增加。反过来,DMD中来自细胞器的Ca 2+外流速率增加可能是NCLX水平显着增加的结果。此外,mdx小鼠的心脏线粒体对MPT孔的开放更具抵抗力,这可能是由于DMD小鼠线粒体膜的微粘度增加所致。同时,推定的MPT孔蛋白水平没有改变。本文讨论了参与钙的运输和积累的线粒体蛋白质组的重排对该器官对DMD的适应性的影响。

更新日期:2020-06-28
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