Departments of Neurology, University of Miami Miller School of Medicine, 1095 NW 14th Terrace, Miami, FL 33136, USA.
J Inherit Metab Dis. 2011 Aug;34(4):941-51. doi: 10.1007/s10545-011-9330-8. Epub 2011 May 4.
Mitochondrial biogenesis is a complex process depending on both nuclear and mitochondrial DNA (mtDNA) transcription regulation to tightly coordinate mitochondrial levels and the cell's energy demand. The energy requirements for a cell to support its metabolic function can change in response to varying physiological conditions, such as, proliferation and differentiation. Therefore, mitochondrial transcription regulation is constantly being modulated in order to establish efficient mitochondrial oxidative metabolism and proper cellular function. The aim of this article is to review the function of major protein factors that are directly involved in the process of mtDNA transcription regulation, as well as, the importance of mitochondrial nucleoid structure and its influence on mtDNA segregation and transcription regulation. Here, we discuss the current knowledge on the molecular mode of action of transcription factors comprising the mitochondrial transcriptional machinery, as well as the action of nuclear receptors on regulatory regions of the mtDNA.
线粒体生物发生是一个复杂的过程，取决于核和线粒体 DNA（mtDNA）转录调控，以紧密协调线粒体水平和细胞的能量需求。细胞支持其代谢功能的能量需求可以根据不同的生理条件（如增殖和分化）而变化。因此，为了建立有效的线粒体氧化代谢和适当的细胞功能，线粒体转录调控不断被调节。本文的目的是综述直接参与 mtDNA 转录调控过程的主要蛋白质因子的功能，以及线粒体核区结构的重要性及其对 mtDNA 分离和转录调控的影响。在这里，我们讨论了组成线粒体转录机制的转录因子的分子作用模式的最新知识，以及核受体对 mtDNA 调控区的作用。
