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Mitochondrial DNA repair and damage tolerance
Abstract
The accurate maintenance of mitochondrial DNA (mtDNA) is required in order for eukaryotic cells to assemble a functional electron transport chain. This independently-maintained genome relies on nuclear-encoded proteins that are imported into the mitochondria to carry out replication and repair processes. Decades of research has made clear that mitochondria employ robust and varied mtDNA repair and damage tolerance mechanisms in order to ensure the proper maintenance of the mitochondrial genome. This review focuses on our current understanding of mtDNA repair and damage tolerance pathways including base excision repair, mismatch repair, homologous recombination, non-homologous end joining, translesion synthesis and mtDNA degradation in both yeast and mammalian systems.
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“Furthermore, SSBs and DSBs generated as products of replication stalling, ROS activity and abortive topoisomerase action may be repaired by either HR or NHEJ [142,143].”
From Focus on UV-Induced DNA Damage and Repair—Disease Relevance and Protective Strategies (2020) · cites this paper as [142]
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Mitochondrial DNA Integrity: Role in Health and Disease
Sharma, P., & Sampath, H. (2019) · Cells · doi:10.3390/cells8020100 · Cited by 281
Obesity, DNA Damage, and Development of Obesity-Related Diseases
Włodarczyk, M., & Nowicka, G. (2019) · International Journal of Molecular Sciences · doi:10.3390/ijms20051146 · Cited by 257
Role of Mitochondria in Radiation Responses: Epigenetic, Metabolic, and Signaling Impacts
Averbeck, D., & Rodriguez‐Lafrasse, C. (2021) · International Journal of Molecular Sciences · doi:10.3390/ijms222011047 · Cited by 262
Zhang, Z., Huang, Q., Zhao, D., Lian, F., Li, X., & Qi, W. (2023) · Frontiers in Endocrinology · doi:10.3389/fendo.2023.1112363 · Cited by 236
The Mitochondrial Response to DNA Damage
Rong, Z., Tu, P., Xu, P., Sun, Y., Yu, F., Tu, N., Guo, L., & Yang, Y. (2021) · Frontiers in Cell and Developmental Biology · doi:10.3389/fcell.2021.669379 · Cited by 191
Metadata
- Authors
- Sia, E. A.
- Affiliations
- University of Rochester (US)
- Corresponding author
- Sia, Elaine A
- ORCID
- Sia, Elaine A
- Venue
- Frontiers in Bioscience
- Publisher
- IMR Press
- Published
- 2017
- Volume
- 22
- Issue
- 5
- Pages
- 920-943
- ISSN
- 1093-4715 (ISSN-L)
- 1093-9946
- DOI
- 10.2741/4525
- OpenAlex
- W2547799867
- Cited by
- 84
- References
- 0
- Citations by year
- 2025: 5 · 2024: 9 · 2023: 7 · 2022: 10 · 2021: 12 · 2020: 25 · 2019: 12 · 2018: 1 · 2017: 3
- FWCI
- 2.77
- Type
- journal article
- Language
- en
- Open access
- closed
- Locations
- Frontiers in bioscience (publishedVersion)
- PubMed (publishedVersion)
- Topics
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › DNA Repair Mechanisms
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Mitochondrial Function and Pathology
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Fungal and yeast genetics research
- Keywords
- mitochondrial DNA, mtDNA repair, damage tolerance, base excision repair, DNA mismatch repair, homologous recombination, non-homologous end joining, translesion synthesis, mitochondrial DNA maintenance, nuclear-encoded mitochondrial proteins, electron transport chain
- MeSH terms
- Animals, DNA Damage, DNA Repair, DNA, Fungal, DNA, Mitochondrial, Humans, Mitochondria, Mutation, Saccharomyces cerevisiae, Genome, Human, Genome, Fungal, Genome, Mitochondrial
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- Sia, E. A. (2017). Mitochondrial DNA repair and damage tolerance. Frontiers in Bioscience. https://doi.org/10.2741/4525
