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IJMS
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Synthesis and Assembly of the Mitochondrially Encoded Proteins
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Synthesis and Assembly of the Mitochondrially Encoded Proteins

A special issue of International Journal of Molecular Sciences (ISSN 1422-0067). This special issue belongs to the section "Molecular Genetics and Genomics".

Deadline for manuscript submissions: closed (30 November 2020) | Viewed by 20350

Special Issue Editors

Dr. Joanna E. Rorbach

E-Mail Website
Guest Editor
MRC Mitochondrial Biology Unit, University of Cambridge, Cambridge Biomedical Campus, Wellcome Trust/MRC Building, Hills Road, Cambridge CB2 0XY, UK
Interests: RNA biology; mitochondria; ribosome biology; cryoEM; proteomics; OXPHOS; protein synthesis
Dr. Isabel Lopez Sanchez

E-Mail Website
Guest Editor
University of Melbourne, Parkville, Australia
Interests: mitochondria; OXPHOS; gene expression; RNA processing; cellular metabolism; neurodegeneration; ageing

Special Issue Information

Dear Colleagues,

The mitochondrial genome encodes 11 mRNAs, 22 tRNAs, and 2 rRNAs, which constitute the core of the mitochondrial protein synthesis machinery. All additional components required for mitochondrial translation, assembly of the newly produced proteins into functional OXPHOS complexes, and regulation of these processes are encoded by the nuclear genome and imported from the cytosol.

In recent years, there have been significant advances in our understanding of the specific factors and mechanisms that mediate these processes. For this Special Issue of IJMS on “Synthesis and Assembly of Mitochondrially Encoded Proteins”, we invite scientists to contribute original articles, mini and full reviews, or perspectives that highlight recent developments in these areas of mitochondrial biology.

Topics of interest include, but are not limited to, the following: mitochondrial RNA maturation, processing, modification, and degradation; mitochondrial ribosome assembly and translation; import and translocation of nuclear DNA-encoded mitochondrial proteins; biogenesis of individual OXPHOS complexes and supercomplex formation; and regulation and signaling in response to metabolic adaptations, mitochondrial damage, and aging.

Dr. Joanna E. Rorbach
Dr. Isabel Lopez Sanchez
Guest Editors

Manuscript Submission Information

Manuscripts should be submitted online at www.mdpi.com by registering and logging in to this website. Once you are registered, click here to go to the submission form. Manuscripts can be submitted until the deadline. All submissions that pass pre-check are peer-reviewed. Accepted papers will be published continuously in the journal (as soon as accepted) and will be listed together on the special issue website. Research articles, review articles as well as short communications are invited. For planned papers, a title and short abstract (about 100 words) can be sent to the Editorial Office for announcement on this website.

Submitted manuscripts should not have been published previously, nor be under consideration for publication elsewhere (except conference proceedings papers). All manuscripts are thoroughly refereed through a single-blind peer-review process. A guide for authors and other relevant information for submission of manuscripts is available on the Instructions for Authors page. International Journal of Molecular Sciences is an international peer-reviewed open access semimonthly journal published by MDPI.

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Keywords

  • mitochondrial RNA maturation
  • mitochondrial RNA processing
  • mitochondrial RNA modification
  • mitochondrial RNA degradation
  • mitochondrial ribosome assembly
  • mitochondrial translation
  • protein import and translocation
  • OXPHOS complexes biogenesis
  • OXPHOS supercomplex formation
  • mitochondrial protein expression regulation and signaling

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Published Papers (3 papers)

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Research

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18 pages, 2244 KiB  
Article
Yeast Mitochondrial Translation Initiation Factor 3 Interacts with Pet111p to Promote COX2 mRNA Translation
by Ivan Chicherin, Sergey Levitskii, Maria V. Baleva, Igor A. Krasheninnikov, Maxim V. Patrushev and Piotr Kamenski
Int. J. Mol. Sci. 2020, 21(10), 3414; https://doi.org/10.3390/ijms21103414 - 12 May 2020
Cited by 3 | Viewed by 2851
Abstract
Mitochondrial genomes code for several core components of respiratory chain complexes. Thus, mitochondrial translation is of great importance for the organelle as well as for the whole cell. In yeast, mitochondrial translation initiation factor 3, Aim23p, is not essential for the organellar protein [...] Read more.
Mitochondrial genomes code for several core components of respiratory chain complexes. Thus, mitochondrial translation is of great importance for the organelle as well as for the whole cell. In yeast, mitochondrial translation initiation factor 3, Aim23p, is not essential for the organellar protein synthesis; however, its absence leads to a significant quantitative imbalance of the mitochondrial translation products. This fact points to a possible specific action of Aim23p on the biosynthesis of some mitochondrial protein species. In this work, we examined such peculiar effects of Aim23p in relation to yeast mitochondrial COX2 mRNA translation. We show that Aim23p is indispensable to this process. According to our data, this is mediated by Aimp23p interaction with the known specific factor of the COX2 mRNA translation, Pet111p. If there is no Aim23p in the yeast cells, an increased amount of Pet111p ensures proper COX2 mRNA translation. Our results demonstrate the additional non-canonical function of initiation factor 3 in yeast mitochondrial translation. Full article
(This article belongs to the Special Issue Synthesis and Assembly of the Mitochondrially Encoded Proteins)
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Review

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32 pages, 2980 KiB  
Review
Human Mitoribosome Biogenesis and Its Emerging Links to Disease
by Maria Isabel G. Lopez Sanchez, Annika Krüger, Dmitrii I. Shiriaev, Yong Liu and Joanna Rorbach
Int. J. Mol. Sci. 2021, 22(8), 3827; https://doi.org/10.3390/ijms22083827 - 7 Apr 2021
Cited by 31 | Viewed by 5587
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 [...] Read more.
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. Full article
(This article belongs to the Special Issue Synthesis and Assembly of the Mitochondrially Encoded Proteins)
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32 pages, 892 KiB  
Review
Mitochondrial OXPHOS Biogenesis: Co-Regulation of Protein Synthesis, Import, and Assembly Pathways
by Jia Xin Tang, Kyle Thompson, Robert W. Taylor and Monika Oláhová
Int. J. Mol. Sci. 2020, 21(11), 3820; https://doi.org/10.3390/ijms21113820 - 28 May 2020
Cited by 83 | Viewed by 11373
Abstract
The assembly of mitochondrial oxidative phosphorylation (OXPHOS) complexes is an intricate process, which—given their dual-genetic control—requires tight co-regulation of two evolutionarily distinct gene expression machineries. Moreover, fine-tuning protein synthesis to the nascent assembly of OXPHOS complexes requires regulatory mechanisms such as translational plasticity [...] Read more.
The assembly of mitochondrial oxidative phosphorylation (OXPHOS) complexes is an intricate process, which—given their dual-genetic control—requires tight co-regulation of two evolutionarily distinct gene expression machineries. Moreover, fine-tuning protein synthesis to the nascent assembly of OXPHOS complexes requires regulatory mechanisms such as translational plasticity and translational activators that can coordinate mitochondrial translation with the import of nuclear-encoded mitochondrial proteins. The intricacy of OXPHOS complex biogenesis is further evidenced by the requirement of many tightly orchestrated steps and ancillary factors. Early-stage ancillary chaperones have essential roles in coordinating OXPHOS assembly, whilst late-stage assembly factors—also known as the LYRM (leucine–tyrosine–arginine motif) proteins—together with the mitochondrial acyl carrier protein (ACP)—regulate the incorporation and activation of late-incorporating OXPHOS subunits and/or co-factors. In this review, we describe recent discoveries providing insights into the mechanisms required for optimal OXPHOS biogenesis, including the coordination of mitochondrial gene expression with the availability of nuclear-encoded factors entering via mitochondrial protein import systems. Full article
(This article belongs to the Special Issue Synthesis and Assembly of the Mitochondrially Encoded Proteins)
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