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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

Received: 　　Accepted:

Corresponding author: 余晨　　E-mail:

DOI: 10.13294/j.aps.2024.0054

Citing This Article：

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).