DNA Polymerase Gamma in Mitochondrial DNA Replication and Repair
نویسندگان
چکیده
منابع مشابه
Replication Pauses of the Wild-Type and Mutant Mitochondrial DNA Polymerase Gamma: A Simulation Study
The activity of polymerase γ is complicated, involving both correct and incorrect DNA polymerization events, exonuclease activity, and the disassociation of the polymerase:DNA complex. Pausing of pol-γ might increase the chance of deletion and depletion of mitochondrial DNA. We have developed a stochastic simulation of pol-γ that models its activities on the level of individual nucleotides for ...
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The human mitochondrial DNA polymerase gamma (Pol-γ) is nuclearly encoded and is responsible for the replication and repair of the mitochondrial genome. Mutations S305R and P1073L in the POLG gene have been reported to be associated with early childhood Alpers syndrome. One patient harboring both mutations as compound heterozygous died at 2 years of age after disease onset at 9 months. Quantita...
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Mitochondrial DNA (mtDNA) polymerase gamma (Polg) is a heterodimeric enzyme containing a Pol I-like catalytic core (PolgA) and an accessory subunit. Mutations in POLGA, affecting the stability of mtDNA, have been identified in several human pathologies such as progressive external ophthalmoplegia and Alpers' syndrome. Extensive literature shows mitochondrial toxicity effects nucleoside analogue...
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Aphidicolin, a known inhibitor of DNA polymerase a, is a potent inhibitor of nuclear DNA synthesis in HeLa cells but has no effect on the replication f mitochondrial DNA. Parallel experiments with mitochondria incubated in ui tm also show no inhibition of DNA synthesis by aphidicolin; however, DNA synthesis in these isolated mitochondria is completely blocked by dideoxycytidine triphosphate, wh...
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ژورنال
عنوان ژورنال: The Scientific World JOURNAL
سال: 2003
ISSN: 1537-744X
DOI: 10.1100/tsw.2003.09