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Review
, 5 (5), a012641

The Maintenance of Mitochondrial DNA Integrity--Critical Analysis and Update

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Review

The Maintenance of Mitochondrial DNA Integrity--Critical Analysis and Update

Mikhail Alexeyev et al. Cold Spring Harb Perspect Biol.

Abstract

DNA molecules in mitochondria, just like those in the nucleus of eukaryotic cells, are constantly damaged by noxious agents. Eukaryotic cells have developed efficient mechanisms to deal with this assault. The process of DNA repair in mitochondria, initially believed nonexistent, has now evolved into a mature area of research. In recent years, it has become increasingly appreciated that mitochondria possess many of the same DNA repair pathways that the nucleus does. Moreover, a unique pathway that is enabled by high redundancy of the mitochondrial DNA and allows for the disposal of damaged DNA molecules operates in this organelle. In this review, we attempt to present a unified view of our current understanding of the process of DNA repair in mitochondria with an emphasis on issues that appear controversial.

Figures

Figure 1.
Figure 1.
The map of human mitochondrial DNA. (OH and OL) Origins of heavy- and light-strand replication, respectively; (ND1–ND6) subunits of NADH dehydrogenase (ETC complex I) subunits 1–6; (COX1–COX3) subunits of cytochrome oxidase subunits 1–3 (ETC complex IV); (ATP6 and ATP8) subunits 6 and 8 of mitochondrial ATPase (complex V); (Cyt b) cytochrome b (complex III).
Figure 2.
Figure 2.
Mitochondrial BER in mammalian cells. Three different subpathways of SP-BER converge at the gap-filling step. SP-BER (left) and LP-BER (right) converge at the nick-sealing step. In addition to FEN1, DNA2 is required for efficient flap processing during LP-BER in mitochondria.

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