Department of Medicine, University of Udine, 33100 Udine, Italy.
Centre of New Technologies, University of Warsaw, 02-097 Warsaw, Poland.
Int J Mol Sci. 2019 May 6;20(9):2221. doi: 10.3390/ijms20092221.
Although the large majority of mitochondrial proteins are nuclear encoded, for their correct functioning mitochondria require the expression of 13 proteins, two rRNA, and 22 tRNA codified by mitochondrial DNA (mtDNA). Once transcribed, mitochondrial RNA (mtRNA) is processed, mito-ribosomes are assembled, and mtDNA-encoded proteins belonging to the respiratory chain are synthesized. These processes require the coordinated spatio-temporal action of several enzymes, and many different factors are involved in the regulation and control of protein synthesis and in the stability and turnover of mitochondrial RNA. In this review, we describe the essential steps of mitochondrial RNA synthesis, maturation, and degradation, the factors controlling these processes, and how the alteration of these processes is associated with human pathologies.
虽然绝大多数线粒体蛋白是由核编码的，但为了正确发挥功能，线粒体还需要表达 13 种蛋白质、2 个 rRNA 和 22 个由线粒体 DNA（mtDNA）编码的 tRNA。线粒体 RNA（mtRNA）转录后，需要经过加工、组装线粒体核糖体，并合成属于呼吸链的 mtDNA 编码蛋白。这些过程需要几种酶的协调时空作用，许多不同的因素参与蛋白质合成的调节和控制，以及线粒体 RNA 的稳定性和周转。在这篇综述中，我们描述了线粒体 RNA 合成、成熟和降解的基本步骤、控制这些过程的因素，以及这些过程的改变如何与人类病理相关。
