Abstract
Mammalian mitochondrial ribosomes (mitoribosomes) synthesize a small subset of proteins, which are essential components of the oxidative phosphorylation machinery. Therefore, their function is of fundamental importance to cellular metabolism. The assembly of mitoribosomes is a complex process that progresses through numerous maturation and protein-binding events coordinated by the actions of several assembly factors. Dysregulation of mitoribosome production is increasingly recognized as a contributor to metabolic and neurodegenerative diseases. In recent years, mutations in multiple components of the mitoribosome assembly machinery have been associated with a range of human pathologies, highlighting their importance to cell function and health. Here, we provide a review of our current understanding of mitoribosome biogenesis, highlighting the key factors involved in this process and the growing number of mutations in genes encoding mitoribosomal RNAs, proteins, and assembly factors that lead to human disease.
Highlights
Mammalian mitochondria contain their own ribosomes that synthesize 13 key subunits of the oxidative phosphorylation (OxPhos) system
While all RNA molecules required for mitochondrial translation, including 11 mitochondrial messenger RNAs, 22 transfer RNAs, and two ribosomal RNAs, are encoded in the mitochondrial DNA, mitoribosomal proteins and auxiliary factors involved in translation are encoded in the nucleus
We summarize our current knowledge of mitoribosome biogenesis, focusing on the roles of assembly factors and provide their emerging links to disease
Summary
Mammalian mitochondria contain their own ribosomes that synthesize 13 key subunits of the oxidative phosphorylation (OxPhos) system. While all RNA molecules required for mitochondrial translation, including 11 mitochondrial messenger RNAs (mt-mRNAs), 22 transfer RNAs (mt-tRNAs), and two ribosomal RNAs (rRNA), are encoded in the mitochondrial DNA (mtDNA), mitoribosomal proteins and auxiliary factors involved in translation are encoded in the nucleus. Mitoribosomes differ significantly from their closest relatives, bacterial 70S ribosomes. This divergence brings unique features into the process of mitochondrial protein synthesis and the mitoribosome biogenesis pathways. The process of assembly of prokaryotic and eukaryotic cytosolic ribosomes is relatively well-studied; many aspects of mitoribosome production remain poorly characterized. We summarize our current knowledge of mitoribosome biogenesis, focusing on the roles of assembly factors and provide their emerging links to disease
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