POLG in the replisome polymerizes the H strand

Stable Identifier
R-HSA-9914364
Type
Reaction [omitted]
Species
Homo sapiens
Compartment
mitochondrial matrix
ReviewStatus
5/5
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POLG in the replisome polymerizes the H strand
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The POLG subunit of mitochondrial DNA polymerase gamma (POLgamma, POLG:POLG2) within the H strand replisome complex polymerizes deoxynucleotide triphosphates to yield a nascent H strand (Johnson et al. 2000, Korhonen et al. 2004, Wanrooij et al. 2008, Plaza-G A. et al. 2023, Wojtaszek et al. 2023). Two subunits of POLG2 bind each POLG subunit and act to increase the DNA binding, replication rate, and processivity of POLG (Lim et al. 1999, Johnson et al. 2000, Fan et al. 2006, Lee et al. 2009, Wojtaszek et al. 2023). The POLG:POLG2 complex (POLgamma) has strand displacement activity that is greatly enhanced by SSBP1 binding the displaced strand (Plaza-G.A. et al. 2023). The H strand replisome contains the DNA helicase TWNK (PEO1), which unwinds DNA in advance of POLgamma.
Literature References
PubMed ID Title Journal Year
18685103 Human mitochondrial RNA polymerase primes lagging-strand DNA synthesis in vitro

Wanrooij, S, Fusté, JM, Farge, G, Shi, Y, Gustafsson, CM, Falkenberg, M

Proc Natl Acad Sci U S A 2008
10608893 The mitochondrial p55 accessory subunit of human DNA polymerase gamma enhances DNA binding, promotes processive DNA synthesis, and confers N-ethylmaleimide resistance

Lim, SE, Longley, MJ, Copeland, WC

J Biol Chem 1999
37592734 Structure-specific roles for PolG2-DNA complexes in maintenance and replication of mitochondrial DNA

Wojtaszek, JL, Hoff, KE, Longley, MJ, Kaur, P, Andres, SN, Wang, H, Williams, RS, Copeland, WC

Nucleic Acids Res 2023
19837034 Structural insight into processive human mitochondrial DNA synthesis and disease-related polymerase mutations

Lee, YS, Kennedy, WD, Yin, YW

Cell 2009
36744436 Mechanism of strand displacement DNA synthesis by the coordinated activities of human mitochondrial DNA polymerase and SSB

Plaza-G A, I, Lemishko, KM, Crespo, R, Truong, TQ, Kaguni, LS, Cao-García, FJ, Ciesielski, GL, Ibarra, B

Nucleic Acids Res 2023
15167897 Reconstitution of a minimal mtDNA replisome in vitro

Korhonen, JA, Pham, XH, Pellegrini, M, Falkenberg, M

EMBO J 2004
16574152 A novel processive mechanism for DNA synthesis revealed by structure, modeling and mutagenesis of the accessory subunit of human mitochondrial DNA polymerase

Fan, L, Kim, S, Farr, CL, Schaefer, KT, Randolph, KM, Tainer, JA, Kaguni, LS

J Mol Biol 2006
10677218 Human mitochondrial DNA polymerase holoenzyme: reconstitution and characterization

Johnson, AA, Tsai, Yc, Graves, SW, Johnson, KA

Biochemistry 2000
Participants
Input
Output
Participates
as an event of
Event Information
Go Biological Process
mitochondrial DNA replication (0006264)
Catalyst Activity

DNA-directed DNA polymerase activity of Mitochondrial H strand replisome with unwound replication fork [mitochondrial matrix]

Physical Entity
Mitochondrial H strand replisome with unwound replication fork [mitochondrial matrix] (Homo sapiens)
Activity
DNA-directed DNA polymerase activity (GO:0003887)
Orthologous Events
POLG in the replisome polymerizes the H strand (Bos taurus)
POLG in the replisome polymerizes the H strand (Canis familiaris)
POLG in the replisome polymerizes the H strand (Drosophila melanogaster)
POLG in the replisome polymerizes the H strand (Gallus gallus)
POLG in the replisome polymerizes the H strand (Mus musculus)
POLG in the replisome polymerizes the H strand (Rattus norvegicus)
POLG in the replisome polymerizes the H strand (Sus scrofa)
POLG in the replisome polymerizes the H strand (Xenopus tropicalis)
Authored
May, B  (2024-06-17)
Reviewed
Wu, CC  (2024-10-27)
Created
May, B  (2024-06-24)
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