当期文章

线粒体铜稳态失衡与纤维化疾病的研究进展

朱赛雅, 刘晶, 余晨^*

上海市同济大学附属同济医院肾内科，上海 200065

摘要

铜离子作为多种酶的辅助因子，参与细胞的多种生命活动。线粒体是细胞中重要的铜储存器。既往有关线粒体铜稳态失衡与血液系统疾病、心肌病以及骨骼肌病变的关联总结较多见，但线粒体铜稳态失衡与纤维化的关联总结较少见。本文以线粒体铜稳态失衡对纤维化的作用及机制为纲，系统阐述了铜吸收和转运、线粒体内铜稳态调节，并聚焦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。

关键词： 线粒体功能; 铜; 铜伴侣蛋白; 纤维化

Research progress on mitochondrial copper homeostasis imbalance and fibrosis diseases

ZHU Sai-Ya, Liu Jing, YU Chen^*

Department of Nephrology, Tongji Hospital, School of Medicine, Tongji University, Shanghai 200065, China

Abstract

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.

Key words: mitochondrial function; copper; copper chaperone protein; fibrosis

收稿日期：　　录用日期：

通讯作者：余晨　　E-mail:

DOI: 10.13294/j.aps.2024.0054

引用本文：

朱赛雅, 刘晶, 余晨. 线粒体铜稳态失衡与纤维化疾病的研究进展[J]. 生理学报 2024; 76 (4): 597-604.

ZHU Sai-Ya, Liu Jing, YU Chen. Research progress on mitochondrial copper homeostasis imbalance and fibrosis diseases. Acta Physiol Sin 2024; 76 (4): 597-604 (in Chinese with English abstract).