Department of Biology, University of Padova, Italy; email:
Nencki Institute of Experimental Biology, Polish Academy of Sciences, Warsaw, Poland; email:
Annu Rev Biophys. 2023 May 9;52:229-254. doi: 10.1146/annurev-biophys-092622-094853.
Mitochondria are involved in multiple cellular tasks, such as ATP synthesis, metabolism, metabolite and ion transport, regulation of apoptosis, inflammation, signaling, and inheritance of mitochondrial DNA. The majority of the correct functioning of mitochondria is based on the large electrochemical proton gradient, whose component, the inner mitochondrial membrane potential, is strictly controlled by ion transport through mitochondrial membranes. Consequently, mitochondrial function is critically dependent on ion homeostasis, the disturbance of which leads to abnormal cell functions. Therefore, the discovery of mitochondrial ion channels influencing ion permeability through the membrane has defined a new dimension of the function of ion channels in different cell types, mainly linked to the important tasks that mitochondrial ion channels perform in cell life and death. This review summarizes studies on animal mitochondrial ion channels with special focus on their biophysical properties, molecular identity, and regulation. Additionally, the potential of mitochondrial ion channels as therapeutic targets for several diseases is briefly discussed.
线粒体参与多种细胞任务，如 ATP 合成、代谢、代谢物和离子转运、细胞凋亡、炎症、信号转导以及线粒体 DNA 的遗传调控。线粒体的大部分正常功能基于大的电化学质子梯度，其组成部分，即线粒体内膜电位，严格受到通过线粒体膜的离子转运控制。因此，线粒体功能严重依赖于离子稳态，离子稳态的紊乱会导致细胞功能异常。因此，发现影响膜离子通透性的线粒体离子通道，定义了离子通道在不同细胞类型中的功能的新维度，主要与线粒体离子通道在细胞生死中发挥的重要任务有关。本综述总结了动物线粒体离子通道的研究，特别关注其生物物理特性、分子特性和调控。此外，简要讨论了线粒体离子通道作为多种疾病治疗靶点的潜力。
