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AAT Bioquest

Where are the proteins involved in mitochondrial DNA replication located?

Posted June 6, 2024


Answer

DNA polymerase γ, mitochondrial RNA polymerase (POLRMT/mtRNAP), Twinkle DNA helicase (c10orf2), and mitochondrial single-stranded DNA binding protein (mtSSB) are the proteins involved in mitochondrial DNA replication. All of these proteins are located within the mitochondrial matrix. Twinkle DNA helicase and POLRMT/mtRNAP are also found in the mitochondrial nucleotides. Additionally, twinkle DNA and DNA polymerase γ are located on the long arm of chromosomes 10 and 15 respectively. Besides these proteins, RNase H1, topoisomerase 3α (Top3α), and DNA ligase III are also essential components of the mitochondrial DNA replication system. These proteins are found in both the nucleus and mitochondria, with DNA ligase III being predominantly found in the nucleus.

Additional resources

The mitochondrial DNA helicase TWINKLE can assemble on a closed circular template and support initiation of DNA synthesis

Mitochondria

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