Zhu Sai-Ya, Liu Jing, Yu Chen
Department of Nephrology, Tongji Hospital, School of Medicine, Tongji University, Shanghai 200065, China.
Sheng Li Xue Bao. 2024 Aug 25;76(4):597-604.
Copper ions serve as co-factors for various enzymes and participate in multiple cellular processes. Mitochondria are essential copper reservoirs within the cell. Previous reviews have extensively summarized the association between mitochondrial copper homeostasis imbalance and hematologic disorders, cardiomyopathies, and skeletal myopathies. However, there is limited information regarding its association with organ fibrosis. This article outlines the role and mechanism of disrupted mitochondrial copper homeostasis in fibrotic diseases, and systematically elaborates copper absorption and transport, as well as the regulation of copper homeostasis within mitochondria. It focuses on the impacts of mitochondrial copper overload and deficiency on fibrotic diseases, and the application of copper chelators as potential anti-fibrotic therapeutic approaches.
铜离子作为多种酶的辅助因子,参与多个细胞过程。线粒体是细胞内重要的铜储存库。以往的综述广泛总结了线粒体铜稳态失衡与血液系统疾病、心肌病和骨骼肌病之间的关联。然而,关于其与器官纤维化的关联信息有限。本文概述了线粒体铜稳态破坏在纤维化疾病中的作用和机制,并系统阐述了铜的吸收和运输,以及线粒体内铜稳态的调节。重点关注线粒体铜过载和缺乏对纤维化疾病的影响,以及铜螯合剂作为潜在抗纤维化治疗方法的应用。