Mitochondria are known as the powerhouse of the cell. For a high-energy–consuming organ, such as the heart, continuous ATP production via oxidative metabolism in the mitochondria is essential. Apart from ATP generation, mitochondria are also key to the regulation of cellular metabolism, calcium homeostasis, and reactive oxygen species (ROS) generation.1 Mitochondrial dysfunction has been strongly implicated in a variety of cardiovascular diseases including ischemic heart disease and heart failure. Furthermore, a large portion of mitochondrial disease patients, a condition caused by mutation of genes for mitochondrial proteins, develop cardiomyopathy indicating a causal role of mitochondria in cardiac dysfunction. Given its significant role in the pathogenesis and the current lack of effective therapy for mitochondrial dysfunction, there is a clear need for discovery and innovation in mitochondrial medicine.2,3 Article, see p 74 It is well established that the fetal heart relies heavily on glycolysis for energy metabolism. A switch from glycolysis to oxidative metabolism in the early postnatal period is associated with explosive mitochondrial biogenesis.4,5 The switch is critical for the postnatal maturation of the heart. Loss of PGC-1α/β (peroxisome proliferator–activated receptor gamma coactivator), the powerful transcriptional regulators of mitochondrial biogenesis, in perinatal and postnatal periods, results in lethal cardiomyopathy.6,7 The role of mitochondria in the embryonic cardiomyocytes is, however, less explored. Recent studies using pluripotent cell–derived cardiomyocytes have suggested intriguing functions of mitochondria beyond energy provision in the regulation of cardiomyocytes maturation.6,8–10 …

中文翻译：

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失败的电厂变成大规模谋杀案

线粒体被称为细胞的动力源。对于心脏等高能量消耗器官，通过线粒体中的氧化代谢连续产生ATP是必不可少的。除了ATP的产生，线粒体也是调节细胞代谢，钙稳态和活性氧（ROS）产生的关键。1线粒体功能障碍与多种心血管疾病密切相关，包括缺血性心脏病和心力衰竭。此外，由线粒体蛋白基因突变引起的大部分线粒体疾病患者发展为心肌病，表明线粒体在心脏功能障碍中起因果作用。鉴于其在发病机理中的重要作用以及当前缺乏针对线粒体功能障碍的有效治疗方法，显然需要发现和创新线粒体药物。2,3条，请参见第74页众所周知，胎儿心脏严重依赖糖酵解来进行能量代谢。出生后早期从糖酵解转变为氧化代谢与线粒体爆炸性生物发生有关[4,5]。该转变对于心脏的出生后成熟至关重要。PGC-1α/β（过氧化物酶体增殖物激活的受体γ辅激活物）的丢失，是围产期和产后时期线粒体生物发生的强大转录调节因子，导致致命的心肌病。6,7然而，线粒体在胚胎心肌细胞中的作用是，探索较少。