Mitochondrial aminoacyl-tRNA synthetases
Mitochondrial aminoacyl-tRNA synthetases
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DOI：
10.1016/bs.enz.2020.07.003
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发表时间：
2020-01-01
期刊：
影响因子：
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通讯作者：
Chihade, Joseph
中科院分区：
文献类型：
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作者：
Chihade, Joseph
在所有真核细胞中，蛋白质合成不仅发生在胞质溶胶中，而且发生在线粒体中。线粒体基因的翻译需要一组氨酰-tRNA合成酶，其中许多通常专门用于细胞器功能。这些酶已经进化出独特的机制来识别tRNA并确保翻译的保真度。人类线粒体合成酶的突变与广泛的致病性表型相关，既突出了它们在维持细胞“发电站”中的作用的重要性，又暗示了额外的细胞作用。
In all eukaryotic cells, protein synthesis occurs not only in the cytosol, but also in the mitochondria. Translation of mitochondrial genes requires a set of aminoacyl-tRNA synthetases, many of which are often specialized for organellar function. These enzymes have evolved unique mechanisms for tRNA recognition and for ensuring fidelity of translation. Mutations of human mitochondrial synthetases are associated with a wide range of pathogenic phenotypes, both highlighting the importance of their role in maintaining the cellular "powerhouse" and suggesting additional cellular roles.