中文摘要：

线粒体转录终止因子（mitochondrial transcription termination factor, MTERF）是一类由核基因编码且高度保守的线粒体DNA结合单体蛋白质，广泛存在于后生动物和植物中．近年来，MTERF蛋白家族对线粒体基因表达调控的作用及其机制研究获得大量成果，对阐明线粒体基因组的表达调控机制及研究人类线粒体疾病等方面有重要的意义．本文综述MTERF蛋白家族4个成员的性质，及其对线粒体DNA复制、转录和翻译的影响，探讨MTERF蛋白家族各成员调控线粒体基因表达的可能机制，展望其对线粒体疾病研究的应用前景．

英文摘要：

Mitochondrial transcription termination factors （MTERFs） are a group of highly conserved mtDNA-binding monomeric proteins that are encoded by nuclear genes. MTERFs are widely found in metazoans and plants. In recent years, a lot of remarkable progress about the regulatory roles of MTERFs in mitochondrial gene expression has been made. These research achievements are very significant for elucidating expression and regulation of mitochondrial genome, and for studying human mitochondrial diseases. This article summarized the characteristics of four MTERF family members and their different regulation roles in mtDNA replication, transcription, and translation. The regulatory mechanism of each MTERF family member in mitochondrial gene expression and their application prospects for research of human mitochondrial diseases was also discussed.