Halmos Tamás, Suba Ilona
1 MAZSIHISZ Szeretetkórház, Metabolikus Ambulancia Budapest Magyarország.
2 Bajcsy-Zsilinszky Kórház és Rendelőintézet, X. Tüdőgondozó Budapest Magyarország.
Orv Hetil. 2022 Aug 28;163(35):1383-1393. doi: 10.1556/650.2022.32552.
The morphological description of mitochondria has been known for nearly two hundred years. Mitochondria are found in all human cells, and a thorough understanding of their function in healthy and pathological conditions was a slow progress. In recent decades, the understanding of this extensive network has accelerated, both in the theoretical field and in clinical practice. Our aim was to review the biogenesis of mitochondria and the diseases caused by their dysfunction, based on the current state of the art. We review the literature to describe the major areas of mitochondrial function, such as ATP production, which is crucial for the energy supply of the body, and the importance of the pyruvate and citric acid cycles, the electron transport chain, oxidative phosphorylation and ROS formation. The function of mitohormesis, which contributes to the body's defences, is described. After a description of mitochondrial dysfunction, we turn to the characterisation of the different mitochondrial pathologies. Having discussed the congenital mitochondrial pathologies, we highlight the fibrosis that severely impairs the function of certain parenchymal organs, heart, liver, kidneys, lungs. We emphasize the importance of cardiac fibrosis, in particular cardiac arrhythmias associated with mitochondrial dysfunction, briefly mentioning the latest therapeutic recommendations. In this context, results are expected from the use of SGLT2 or combined SGLT1/2 inhibitor. The role of this system in type 1 diabetes mellitus and in the development of insulin resistance and type 2 diabetes is mentioned as well. We outline the role of mitochondrial dysfunction in the development of nerodegenerative diseases. The importance of exercise, antioxidant therapy, cardiolipin protection, enhancement of mitochondrial biogenesis, use of sodium-glucose co-transporter inhibitors, and - underlined - the recently introduced hopeful mitochondrial transplantation in the management of these pathologies are highlighted. The mitochondrial system is not only an energy centre but also regulates the function of all our vital organs. We have shown that in the case of mitochondrial dysfunction, the function of our vital organs becomes critical due to the fibrosis that develops. However, we do not address the oncological aspects of mitochondria and mitochondrial dysfunction, as this is beyond the scope of this article. The aim of this work is primarily to explore the multiple interrelationships of this system, to deepen our knowledge and to use it for the better care of our patients. Some believe that mitochondria will determine the future of medicine.
线粒体的形态学描述已有近两百年的历史。线粒体存在于所有人体细胞中，而全面了解其在健康和病理状态下的功能进展缓慢。近几十年来，无论是在理论领域还是临床实践中，对这个庞大网络的认识都加速了。我们的目的是根据当前的技术水平，综述线粒体的生物发生及其功能障碍所引发的疾病。我们查阅文献以描述线粒体功能的主要领域，如对身体能量供应至关重要的ATP生成，以及丙酮酸和柠檬酸循环、电子传递链、氧化磷酸化和活性氧生成的重要性。还描述了有助于身体防御的线粒体应激反应的功能。在描述线粒体功能障碍后，我们转向不同线粒体疾病的特征描述。在讨论先天性线粒体疾病后，我们着重指出严重损害某些实质器官（心脏、肝脏、肾脏、肺）功能的纤维化。我们强调心脏纤维化的重要性，特别是与线粒体功能障碍相关的心律失常，并简要提及最新的治疗建议。在这种背景下，预计使用钠-葡萄糖协同转运蛋白2（SGLT2）或联合使用SGLT1/2抑制剂会有效果。还提到了该系统在1型糖尿病以及胰岛素抵抗和2型糖尿病发展中的作用。我们概述了线粒体功能障碍在神经退行性疾病发展中的作用。强调了运动、抗氧化治疗、心磷脂保护、增强线粒体生物发生、使用钠-葡萄糖协同转运蛋白抑制剂的作用，以及——着重指出——最近引入的有望用于这些疾病治疗的线粒体移植。线粒体系统不仅是一个能量中心，还调节我们所有重要器官的功能。我们已经表明，在线粒体功能障碍的情况下，由于发生的纤维化，我们重要器官的功能变得至关重要。然而，我们不涉及线粒体和线粒体功能障碍的肿瘤学方面，因为这超出了本文的范围。这项工作的目的主要是探索这个系统的多种相互关系，加深我们的认识，并将其用于更好地照顾我们的患者。有些人认为线粒体将决定医学的未来。
