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Open Access Highly Accessed Review

Mitochondrial-associated metabolic disorders: foundations, pathologies and recent progress

Joseph McInnes

Author Affiliations

School of Engineering and Science, Research Center MOLIFE – Molecular Life Science, Jacobs University Bremen, Campus Ring 1, Research II, Room 120, Bremen D-28759, Germany

VIB Center for Biology of Disease, Katholieke Universiteit Leuven, Leuven, Belgium

Center for Human Genetics and Leuven Research Institute for Neurodegenerative Diseases (LIND), Katholieke Universiteit Leuven, Leuven, Belgium

Nutrition & Metabolism 2013, 10:63  doi:10.1186/1743-7075-10-63

Published: 12 October 2013

Abstract

Research in the last decade has revolutionized the way in which we view mitochondria. Mitochondria are no longer viewed solely as cellular powerhouses; rather, mitochondria are now understood to be vibrant, mobile structures, constantly undergoing fusion and fission, and engaging in intimate interactions with other cellular compartments and structures. Findings have implicated mitochondria in a wide variety of cellular processes and molecular interactions, such as calcium buffering, lipid flux, and intracellular signaling. As such, it does not come as a surprise that an increasing number of human pathologies have been associated with functional defects in mitochondria. The difficulty in understanding and treating human pathologies caused by mitochondrial dysfunction arises from the complex relationships between mitochondria and other cellular processes, as well as the genetic background of such diseases. This review attempts to provide a summary of the background knowledge and recent developments in mitochondrial processes relating to mitochondrial-associated metabolic diseases arising from defects or deficiencies in mitochondrial function, as well as insights into current and future avenues for investigation.

Keywords:
Mitochondria; mtDNA; Metabolism; Disease