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Cells 2019, 8(2), 100;

Mitochondrial DNA Integrity: Role in Health and Disease

Department of Nutritional Sciences and New Jersey Institute for Food, Nutrition, and Health, Rutgers University, New Brunswick, NJ 08901, USA
Author to whom correspondence should be addressed.
Received: 31 December 2018 / Revised: 25 January 2019 / Accepted: 28 January 2019 / Published: 29 January 2019
(This article belongs to the Special Issue Mitochondrial Biology in Health and Disease)
Full-Text   |   PDF [758 KB, uploaded 29 January 2019]   |  


As the primary cellular location for respiration and energy production, mitochondria serve in a critical capacity to the cell. Yet, by virtue of this very function of respiration, mitochondria are subject to constant oxidative stress that can damage one of the unique features of this organelle, its distinct genome. Damage to mitochondrial DNA (mtDNA) and loss of mitochondrial genome integrity is increasingly understood to play a role in the development of both severe early-onset maladies and chronic age-related diseases. In this article, we review the processes by which mtDNA integrity is maintained, with an emphasis on the repair of oxidative DNA lesions, and the cellular consequences of diminished mitochondrial genome stability. View Full-Text
Keywords: mitochondrial DNA; base excision repair; metabolic syndrome; neurodegenerative diseases; aging mitochondrial DNA; base excision repair; metabolic syndrome; neurodegenerative diseases; aging

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Sharma, P.; Sampath, H. Mitochondrial DNA Integrity: Role in Health and Disease. Cells 2019, 8, 100.

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