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Int. J. Mol. Sci. 2015, 16(5), 10870-10887; doi:10.3390/ijms160510870

Borrowing Nuclear DNA Helicases to Protect Mitochondrial DNA

Department of Radiation Biology, Beckman Research Institute, City of Hope, Duarte, CA 91010-3000, USA
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Author to whom correspondence should be addressed.
Academic Editors: Jaime M. Ross and Giuseppe Coppotelli
Received: 7 April 2015 / Revised: 9 May 2015 / Accepted: 11 May 2015 / Published: 13 May 2015
(This article belongs to the Special Issue Mitochondrial Dysfunction in Ageing and Diseases)
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Abstract

In normal cells, mitochondria are the primary organelles that generate energy, which is critical for cellular metabolism. Mitochondrial dysfunction, caused by mitochondrial DNA (mtDNA) mutations or an abnormal mtDNA copy number, is linked to a range of human diseases, including Alzheimer’s disease, premature aging‎ and cancer. mtDNA resides in the mitochondrial lumen, and its duplication requires the mtDNA replicative helicase, Twinkle. In addition to Twinkle, many DNA helicases, which are encoded by the nuclear genome and are crucial for nuclear genome integrity, are transported into the mitochondrion to also function in mtDNA replication and repair. To date, these helicases include RecQ-like helicase 4 (RECQ4), petite integration frequency 1 (PIF1), DNA replication helicase/nuclease 2 (DNA2) and suppressor of var1 3-like protein 1 (SUV3). Although the nuclear functions of some of these DNA helicases have been extensively studied, the regulation of their mitochondrial transport and the mechanisms by which they contribute to mtDNA synthesis and maintenance remain largely unknown. In this review, we attempt to summarize recent research progress on the role of mammalian DNA helicases in mitochondrial genome maintenance and the effects on mitochondria-associated diseases. View Full-Text
Keywords: mitochondrial DNA; DNA replication; DNA repair; RECQ4; Twinkle; PIF1; DNA2; SUV3 mitochondrial DNA; DNA replication; DNA repair; RECQ4; Twinkle; PIF1; DNA2; SUV3
This is an open access article distributed under the Creative Commons Attribution License which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. (CC BY 4.0).

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Ding, L.; Liu, Y. Borrowing Nuclear DNA Helicases to Protect Mitochondrial DNA. Int. J. Mol. Sci. 2015, 16, 10870-10887.

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