Int. J. Mol. Sci. 2011, 12(10), 7114-7162; doi:10.3390/ijms12107114
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Manganese Superoxide Dismutase: Guardian of the Powerhouse

Graduate Center for Toxicology, University of Kentucky, 454 HSRB, 1095 VA Drive, Lexington, KY 40536, USA
* Author to whom correspondence should be addressed.
Received: 8 August 2011; in revised form: 28 September 2011 / Accepted: 8 October 2011 / Published: 21 October 2011
(This article belongs to the Special Issue Oxidative Stress and Mitochondria)
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Abstract: The mitochondrion is vital for many metabolic pathways in the cell, contributing all or important constituent enzymes for diverse functions such as β-oxidation of fatty acids, the urea cycle, the citric acid cycle, and ATP synthesis. The mitochondrion is also a major site of reactive oxygen species (ROS) production in the cell. Aberrant production of mitochondrial ROS can have dramatic effects on cellular function, in part, due to oxidative modification of key metabolic proteins localized in the mitochondrion. The cell is equipped with myriad antioxidant enzyme systems to combat deleterious ROS production in mitochondria, with the mitochondrial antioxidant enzyme manganese superoxide dismutase (MnSOD) acting as the chief ROS scavenging enzyme in the cell. Factors that affect the expression and/or the activity of MnSOD, resulting in diminished antioxidant capacity of the cell, can have extraordinary consequences on the overall health of the cell by altering mitochondrial metabolic function, leading to the development and progression of numerous diseases. A better understanding of the mechanisms by which MnSOD protects cells from the harmful effects of overproduction of ROS, in particular, the effects of ROS on mitochondrial metabolic enzymes, may contribute to the development of novel treatments for various diseases in which ROS are an important component.
Keywords: manganese superoxide dismutase; mitochondria; reactive oxygen species; oxidative stress; metabolism; cancer; cardiovascular disease; neurodegenerative disorders

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MDPI and ACS Style

Holley, A.K.; Bakthavatchalu, V.; Velez-Roman, J.M.; St. Clair, D.K. Manganese Superoxide Dismutase: Guardian of the Powerhouse. Int. J. Mol. Sci. 2011, 12, 7114-7162.

AMA Style

Holley AK, Bakthavatchalu V, Velez-Roman JM, St. Clair DK. Manganese Superoxide Dismutase: Guardian of the Powerhouse. International Journal of Molecular Sciences. 2011; 12(10):7114-7162.

Chicago/Turabian Style

Holley, Aaron K.; Bakthavatchalu, Vasudevan; Velez-Roman, Joyce M.; St. Clair, Daret K. 2011. "Manganese Superoxide Dismutase: Guardian of the Powerhouse." Int. J. Mol. Sci. 12, no. 10: 7114-7162.

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