首页|线粒体铜稳态失衡与纤维化疾病的研究进展

线粒体铜稳态失衡与纤维化疾病的研究进展

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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。
Research progress on mitochondrial copper homeostasis imbalance and fibrosis diseases
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.

mitochondrial functioncoppercopper chaperone proteinfibrosis

朱赛雅、刘晶、余晨

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上海市同济大学附属同济医院肾内科,上海 200065

线粒体功能 铜伴侣蛋白 纤维化

National Natural Science Foundation of ChinaNational Natural Science Foundation of China

8187360982170696

2024

生理学报
中国科学院上海生命科学研究院,中国生理学会

生理学报

CSTPCD北大核心
影响因子:0.864
ISSN:0371-0874
年,卷(期):2024.76(4)