Center for Mitochondrial Research and Medicine, Kaohsiung Chang Gung Memorial Hospital, Kaohsiung 833401, Taiwan.
Center for Mitochondrial Research and Medicine, Kaohsiung Chang Gung Memorial Hospital, Kaohsiung 833401, Taiwan; Department of Anesthesiology, Kaohsiung Chang Gung Memorial Hospital, Chang Gung University College of Medicine, Kaohsiung 833401, Taiwan.
Mitochondrion. 2024 May;76:101856. doi: 10.1016/j.mito.2024.101856. Epub 2024 Feb 24.
Mitochondria are important for maintaining cellular energy metabolism and regulating cellular senescence. Mitochondrial DNA (mtDNA) encodes subunits of the OXPHOS complexes which are essential for cellular respiration and energy production. Meanwhile, mtDNA variants have been associated with the pathogenesis of neurodegenerative diseases, including MELAS, for which no effective treatment has been developed. To alleviate the pathological conditions involved in mitochondrial disorders, mitochondria transfer therapy has shown promise. Wharton's jelly mesenchymal stem cells (WJMSCs) have been identified as suitable mitochondria donors for mitochondria-defective cells, wherein mitochondrial functions can be rescued. Miro1 participates in mitochondria trafficking by anchoring mitochondria to microtubules. In this study, we identified Miro1 over-expression as a factor that could help to enhance the efficiency of mitochondrial delivery. More specifically, we reveal that Miro1 over-expressed WJMSCs significantly improved intercellular communications, cell proliferation rates, and mitochondrial membrane potential, while restoring mitochondrial bioenergetics in mitochondria-defective fibroblasts. Furthermore, Miro1 over-expressed WJMSCs decreased rates of induced apoptosis and ROS production in MELAS fibroblasts; although, Miro1 over-expression did not rescue mtDNA mutation ratios nor mitochondrial biogenesis. This study presents a potentially novel therapeutic strategy for treating mitochondrial encephalomyopathy, lactic acidosis and stroke-like episodes (MELAS), and other diseases associated with dysfunctional mitochondria, while the pathophysiological relevance of our results should be further verified by animal models and clinical studies.
线粒体对于维持细胞能量代谢和调节细胞衰老非常重要。线粒体 DNA(mtDNA)编码 OXPHOS 复合物的亚基,这些亚基对于细胞呼吸和能量产生至关重要。同时,mtDNA 变体与神经退行性疾病的发病机制有关,包括 MELAS,目前还没有开发出有效的治疗方法。为了缓解与线粒体疾病相关的病理状况,线粒体转移治疗显示出了希望。Wharton 果冻间充质干细胞(WJMSCs)已被鉴定为适合线粒体缺陷细胞的线粒体供体,其中可以挽救线粒体功能。Miro1 通过将线粒体锚定在微管上来参与线粒体运输。在这项研究中,我们确定 Miro1 的过表达是一种可以帮助提高线粒体传递效率的因素。更具体地说,我们揭示了过表达 Miro1 的 WJMSCs 可显著改善细胞间通讯、细胞增殖率和线粒体膜电位,同时恢复线粒体缺陷成纤维细胞中的线粒体生物能。此外,过表达 Miro1 的 WJMSCs 降低了 MELAS 成纤维细胞中诱导的细胞凋亡和 ROS 产生的速率;尽管 Miro1 的过表达并没有挽救 mtDNA 突变比例或线粒体生物发生。这项研究为治疗线粒体脑肌病、乳酸酸中毒和卒中样发作(MELAS)以及其他与功能失调的线粒体相关的疾病提供了一种潜在的新治疗策略,而我们研究结果的病理生理学相关性应该通过动物模型和临床研究进一步验证。