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线粒体功能障碍:各种模式生物揭示的遗传和细胞影响。

Mitochondrial Dysfunctions: Genetic and Cellular Implications Revealed by Various Model Organisms.

机构信息

Institute of Biology, College of Natural Sciences, University of Rzeszow, 35-959 Rzeszow, Poland.

出版信息

Genes (Basel). 2024 Sep 1;15(9):1153. doi: 10.3390/genes15091153.

Abstract

Mitochondria play a crucial role in maintaining the energy status and redox homeostasis of eukaryotic cells. They are responsible for the metabolic efficiency of cells, providing both ATP and intermediate metabolic products. They also regulate cell survival and death under stress conditions by controlling the cell response or activating the apoptosis process. This functional diversity of mitochondria indicates their great importance for cellular metabolism. Hence, dysfunctions of these structures are increasingly recognized as an element of the etiology of many human diseases and, therefore, an extremely promising therapeutic target. Mitochondrial dysfunctions can be caused by mutations in both nuclear and mitochondrial DNA, as well as by stress factors or replication errors. Progress in knowledge about the biology of mitochondria, as well as the consequences for the efficiency of the entire organism resulting from the dysfunction of these structures, is achieved through the use of model organisms. They are an invaluable tool for analyzing complex cellular processes, leading to a better understanding of diseases caused by mitochondrial dysfunction. In this work, we review the most commonly used model organisms, discussing both their advantages and limitations in modeling fundamental mitochondrial processes or mitochondrial diseases.

摘要

线粒体在维持真核细胞的能量状态和氧化还原平衡方面起着至关重要的作用。它们负责细胞的代谢效率,提供 ATP 和中间代谢产物。在应激条件下,它们还通过控制细胞反应或激活细胞凋亡过程来调节细胞的存活和死亡。线粒体的这种功能多样性表明它们对细胞代谢非常重要。因此,这些结构的功能障碍越来越被认为是许多人类疾病病因的一个因素,因此也是一个极具前景的治疗靶点。线粒体功能障碍可由核 DNA 和线粒体 DNA 的突变以及应激因素或复制错误引起。通过使用模式生物,可以更好地了解线粒体的生物学以及这些结构功能障碍对整个生物体效率的影响,从而推动对线粒体生物学的认识。它们是分析复杂细胞过程的宝贵工具,有助于更好地理解由线粒体功能障碍引起的疾病。在这项工作中,我们综述了最常用的模式生物,讨论了它们在模拟基本的线粒体过程或线粒体疾病方面的优缺点。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1b53/11431519/eb8fbfb8705d/genes-15-01153-g001.jpg

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