关键词: iron–sulfur cluster mitochondrial disease mitochondrial ribosome mitochondrial translation mitoribosome assembly redox sensing

来  源:   DOI:10.1002/2211-5463.13844

Abstract:
Mitoribosome biogenesis is a complex process involving RNA elements encoded in the mitochondrial genome and mitoribosomal proteins typically encoded in the nuclear genome. This process is orchestrated by extra-ribosomal proteins, nucleus-encoded assembly factors, which play roles across all assembly stages to coordinate ribosomal RNA processing and maturation with the sequential association of ribosomal proteins. Both biochemical studies and recent cryo-EM structures of mammalian mitoribosomes have provided insights into their assembly process. In this article, we will briefly outline the current understanding of mammalian mitoribosome biogenesis pathways and the factors involved. Special attention is devoted to the recent identification of iron-sulfur clusters as structural components of the mitoribosome and a small subunit assembly factor, the existence of redox-sensitive cysteines in mitoribosome proteins and assembly factors, and the role they may play as redox sensor units to regulate mitochondrial translation under stress.
摘要:
有丝分裂体生物发生是一个复杂的过程,涉及线粒体基因组中编码的RNA元件和核基因组中通常编码的有丝分裂体蛋白。这个过程是由核糖体外蛋白精心策划的,核编码的组装因子,它们在所有组装阶段发挥作用,以协调核糖体RNA的加工和成熟与核糖体蛋白的顺序关联。哺乳动物线粒体的生化研究和最近的低温EM结构都提供了对其组装过程的见解。在这篇文章中,我们将简要概述目前对哺乳动物有丝分裂体生物发生途径和相关因素的理解。特别注意最近将铁硫簇鉴定为线粒体的结构成分和小的亚基组装因子,有丝分裂体蛋白和组装因子中氧化还原敏感半胱氨酸的存在,以及它们可能作为氧化还原传感器单元在压力下调节线粒体翻译的作用。
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