线粒体细胞色素C氧化酶(mitochondrial cytochrome coxidase，mt-COX，E .C .1.9.3.1)是线粒体呼吸链的复合物Ⅳ(complex IV)，是呼吸链末端的限速酶，参与能量供应、细胞凋亡、新陈代谢、活性氧产生等重要的生理过程。由于哺乳动物中95%的氧是通过mt-COX催化处理后被利用的，mt-COX在能量产生与调节中起着重要作用，成为研究热点。研究表明，细胞色素C氧化酶异常涉及多种疾病，尤其mt-COXI是其核心基团，深入研究具有重要意义。文章就线粒体细胞色素C氧化酶I的研究进展及其与相关疾病的关系进行综述。“,”Mitochondrial cytochrome C oxidase (mt-COX, E .C .1.9.3.1) is the respiratory chain complex Ⅳ in mitochondria, which is the rate limiting enzyme at the end of respiratory chain, and participates in the important physiological processes such as energy supply, cell apoptosis, metabolism, active oxygen production, etc. Since 95% of oxygen in mammals is utilized by catalytic treatment of mt-cox, mt-cox plays an important role in energy production and regulation and becomes a research hotspot. The research shows that the abnormality of cytochrome C oxidase is related to many diseases, especially mt-coxI, which is the core group of cytochrome C oxidase. This review summarized the research progress and related diseases of mitochondrial cytochrome C oxidase I.