线粒体基因转录调控机制与人类疾病

Mitochondrial gene transcription regulation mechanism and human diseases

人类的线粒体DNA编码了37个基因,这些基因在氧化磷酸化作用中都发挥着重要的作用,而他们表达的失调与人类的疾病和衰老有着密切的联系。除了线粒体基因编码的13个mRNA、12S rRNA、16S rRNA和22个tRNA之外,其他调控线粒体基因的转录、翻译及RNA修饰的因子都由细胞核编码而后转运至线粒体基质中。本文详细讨论了线粒体基因转录的调控机制,并突出了这些调控因子与人类疾病存在的联系。

Human mitochondrial DNA encodes 37 genes that play an important role in oxidative phosphorylation,the dysregulation of which is closely linked to human diseases and aging.In addition to the 13 mRNAs,12S rRNAs,16S rRNAs,and 22 tRNAs encoded by mitochondrial DNA,other factors that regulate the transcription,translation,and RNA editing of mitochondrial genes are all encoded by the nucleus and then transported into the mitochondrial matrix.This paper discusses in detail the regulatory mechanism of mitochondrial gene transcription and highlight the relationship between these regulatory factors and human diseases.