The DOI matches its Crossref record; 156 of the 156 references it cites were found in a registry or an index.
DNA Polymerase γ, The Mitochondrial Replicase
Abstract
DNA polymerase (pol) gamma is the sole DNA polymerase in animal mitochondria. Biochemical and genetic evidence document a key role for pol gamma in mitochondrial DNA replication, and whereas DNA repair and recombination were thought to be limited or absent in animal mitochondria, both have been demonstrated in recent years. Thus, the mitochondrial replicase is also apparently responsible for the relevant DNA synthetic reactions in these processes. Pol gamma comprises a catalytic core in a heterodimeric complex with an accessory subunit. The two-subunit holoenzyme is an efficient and processive polymerase, which exhibits high fidelity in nucleotide selection and incorporation while proofreading errors with its intrinsic 3' 5' exonuclease. Incorporation of nucleotide analogs followed by proofreading failure leads to mitochondrial toxicity in antiviral therapy, and misincorporation during DNA replication leads to mitochondrial mutagenesis and dysfunction. This review describes our current understanding of pol gamma biochemistry and biology, and it introduces other key proteins that function at the mitochondrial DNA replication fork.
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“The catalytic subunit has DNA polymerase, 3′→5′ exonuclease and 5′-dRP lyase activities (Graziewicz et al., 2006; Kaguni, 2004).”
From Role of the mitochondrial DNA replication machinery in mitochondrial DNA mutagenesis, aging and age-related diseases (2017) · cites this paper as Kaguni, 2004
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Gustafsson, C. M., Falkenberg, M., & Larsson, N. (2016) · Annual Review of Biochemistry · doi:10.1146/annurev-biochem-060815-014402 · Cited by 688
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Metadata
- Authors
- Kaguni, L. S.
- Affiliations
- Michigan State University (US)
- Corresponding author
- Kaguni, Laurie S.
- Venue
- Annual Review of Biochemistry
- Publisher
- Annual Reviews
- Published
- 2004-06
- Volume
- 73
- Issue
- 1
- Pages
- 293-320
- ISSN
- 0066-4154 (ISSN-L)
- 1545-4509
- OpenAlex
- W2128968369
- Cited by
- 414
- References
- 156
- Citations by year
- 2026: 8 · 2025: 13 · 2024: 11 · 2023: 12 · 2022: 13 · 2021: 12 · 2020: 15 · 2019: 10 · 2018: 11 · 2017: 16 · 2016: 23 · 2015: 16 · 2014: 16 · 2013: 21 · 2012: 26
- FWCI
- 1.40
- Type
- journal article
- Language
- en
- Open access
- closed
- Locations
- Annual Review of Biochemistry (publishedVersion)
- PubMed (publishedVersion)
- Funders
- American Cancer Society
- National Institutes of Health (5r01hl059656-03, 5r01gm034042-03)
- National Heart, Lung, and Blood Institute (HL59656)
- National Institute of General Medical Sciences (R01GM045295, GM34042, GM45295)
- Topics
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Mitochondrial Function and Pathology
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › DNA Repair Mechanisms
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › DNA and Nucleic Acid Chemistry
- Keywords
- DNA polymerase gamma, mitochondrial DNA replication, mitochondrial DNA repair, DNA recombination, nucleotide misincorporation, mitochondrial mutagenesis, mitochondrial dysfunction
- MeSH terms
- DNA Polymerase gamma, Amino Acid Sequence, Animals, DNA-Directed DNA Polymerase, DNA Replication, Exonucleases, Humans, Mitochondria, Models, Molecular, Molecular Sequence Data, Mutagenesis, Sequence Homology, Amino Acid, Protein Subunits, History, 20th Century
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