关键词: RNA mitochondria mitochondrial disease ribosomes translation

来  源:   DOI:10.1016/j.tcb.2024.05.001

Abstract:
Mitochondria rely on coordinated expression of their own mitochondrial DNA (mtDNA) with that of the nuclear genome for their biogenesis. The bacterial ancestry of mitochondria has given rise to unique and idiosyncratic features of the mtDNA and its expression machinery that can be specific to different organisms. In animals, the mitochondrial protein synthesis machinery has acquired many new components and mechanisms over evolution. These include several new ribosomal proteins, new stop codons and ways to recognise them, and new mechanisms to deliver nascent proteins into the mitochondrial inner membrane. Here we describe the mitochondrial protein synthesis machinery in mammals and its unique mechanisms of action elucidated to date and highlight the technologies poised to reveal the next generation of discoveries in mitochondrial translation.
摘要:
线粒体依靠自身线粒体DNA(mtDNA)与核基因组的协调表达来进行生物发生。线粒体的细菌血统已经产生了mtDNA及其表达机制的独特和特质特征,这些特征可能对不同的生物体具有特异性。在动物中,线粒体蛋白质合成机制在进化过程中获得了许多新的成分和机制。这些包括几种新的核糖体蛋白,新的停止密码子和识别它们的方法,以及将新生蛋白输送到线粒体内膜的新机制。在这里,我们描述了哺乳动物的线粒体蛋白质合成机制及其迄今为止阐明的独特作用机制,并强调了有望揭示线粒体翻译中下一代发现的技术。
公众号