Cited by 415Mitochondrial Function and PathologyDNA Repair MechanismsDNA and Nucleic Acid Chemistry

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DNA Polymerase γ, The Mitochondrial Replicase

Kaguni, Laurie S.·2004·Annual Review of Biochemistry

DOI 10.1146/annurev.biochem.72.121801.161455

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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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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
DOI: 10.1146/annurev.biochem.72.121801.161455
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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DNA Polymerase γ, The Mitochondrial Replicase

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