Mitochondrial transcription and translation: overview.
Mitochondrial transcription and translation: overview.
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DOI：
10.1042/ebc20170102
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发表时间：
2018-07-20
影响因子：
6.4
通讯作者：
Minczuk M
中科院分区：
文献类型：
--
作者：
D'Souza AR;Minczuk M
线粒体是细胞内三磷酸腺苷的主要来源。细胞器内膜中的五个多亚单位复合体参与了ATP产生所需的氧化磷酸化。这些复合体的13个亚基由线粒体基因组编码，通常被称为mtDNA。因此，线粒体DNA的表达对氧化磷酸化复合体的组装和功能至关重要。线粒体DNA基因表达调控机制的缺陷与这些复合体的组装缺陷有关，从而导致线粒体疾病。最近，许多参与这些过程的因素已经被识别和表征，这使得人们对线粒体疾病的基础机制有了更深入的了解。
Mitochondria are the major source of ATP in the cell. Five multi-subunit complexes in the inner membrane of the organelle are involved in the oxidative phosphorylation required for ATP production. Thirteen subunits of these complexes are encoded by the mitochondrial genome often referred to as mtDNA. For this reason, the expression of mtDNA is vital for the assembly and functioning of the oxidative phosphorylation complexes. Defects of the mechanisms regulating mtDNA gene expression have been associated with deficiencies in assembly of these complexes, resulting in mitochondrial diseases. Recently, numerous factors involved in these processes have been identified and characterized leading to a deeper understanding of the mechanisms that underlie mitochondrial diseases.