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Mitochondrial OXPHOS Biogenesis: Co-Regulation of Protein Synthesis, Import, and Assembly Pathways

Human Mitoribosome Biogenesis and Its Emerging Links to Disease

Centre for Eye Research Australia, Royal Victorian Eye and Ear Hospital, 32 Gisborne Street, East Melbourne, VIC 3002, Australia
Department of Medical Biochemistry and Biophysics, Division of Molecular Metabolism, Karolinska Institutet, Solnavägen 9, 171 65 Stockholm, Sweden
Max Planck Institute Biology of Ageing—Karolinska Institutet Laboratory, Karolinska Institutet, 171 65 Stockholm, Sweden
Authors to whom correspondence should be addressed.
Joint first authors.
Academic Editor: Yves Henry
Int. J. Mol. Sci. 2021, 22(8), 3827;
Received: 5 March 2021 / Revised: 23 March 2021 / Accepted: 24 March 2021 / Published: 7 April 2021
(This article belongs to the Special Issue Synthesis and Assembly of the Mitochondrially Encoded Proteins)
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. View Full-Text
Keywords: mitochondria; mitoribosome; assembly factors; rRNA; mitochondrial disease mitochondria; mitoribosome; assembly factors; rRNA; mitochondrial disease
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MDPI and ACS Style

Lopez Sanchez, M.I.G.; Krüger, A.; Shiriaev, D.I.; Liu, Y.; Rorbach, J. Human Mitoribosome Biogenesis and Its Emerging Links to Disease. Int. J. Mol. Sci. 2021, 22, 3827.

AMA Style

Lopez Sanchez MIG, Krüger A, Shiriaev DI, Liu Y, Rorbach J. Human Mitoribosome Biogenesis and Its Emerging Links to Disease. International Journal of Molecular Sciences. 2021; 22(8):3827.

Chicago/Turabian Style

Lopez Sanchez, Maria I.G., Annika Krüger, Dmitrii I. Shiriaev, Yong Liu, and Joanna Rorbach. 2021. "Human Mitoribosome Biogenesis and Its Emerging Links to Disease" International Journal of Molecular Sciences 22, no. 8: 3827.

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