Next Article in Journal
Potential Anti-Cancer Activities and Mechanisms of Costunolide and Dehydrocostuslactone
Next Article in Special Issue
Mitochondria-Derived Reactive Oxygen Species Play an Important Role in Doxorubicin-Induced Platelet Apoptosis
Previous Article in Journal
Prediction of Protein–Protein Interactions with Clustered Amino Acids and Weighted Sparse Representation
Previous Article in Special Issue
Skeletal Muscle Mitochondrial Energetic Efficiency and Aging
Open AccessReview

Borrowing Nuclear DNA Helicases to Protect Mitochondrial DNA

by Lin Ding and Yilun Liu *
Department of Radiation Biology, Beckman Research Institute, City of Hope, Duarte, CA 91010-3000, USA
Author to whom correspondence should be addressed.
Academic Editors: Jaime M. Ross and Giuseppe Coppotelli
Int. J. Mol. Sci. 2015, 16(5), 10870-10887;
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)
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
Show Figures

Figure 1

MDPI and ACS Style

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

Show more citation formats Show less citations formats

Article Access Map by Country/Region

Only visits after 24 November 2015 are recorded.
Back to TopTop