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Journal of Immunology Research
Volume 2014, Article ID 164309, 8 pages
Review Article

Powering the Immune System: Mitochondria in Immune Function and Deficiency

1Department of Neurology, Massachusetts General Hospital, Boston, MA 02114, USA
2Department of Neuroscience, Rehabilitation, Ophthalmology, Genetics, Maternal and Child Health (DINOGMI), University of Genoa, Genoa, 16147 Genova, Italy
3Division of Allergy and Immunology, Department of Pediatrics, Massachusetts General Hospital for Children, Boston, MA 02114, USA
4Novartis Institute for Research in Biomedicine, Basel, Switzerland

Received 16 June 2014; Revised 20 August 2014; Accepted 25 August 2014; Published 21 September 2014

Academic Editor: Luca Gattinoni

Copyright © 2014 Melissa A. Walker et al. 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.


Mitochondria are critical subcellular organelles that are required for several metabolic processes, including oxidative phosphorylation, as well as signaling and tissue-specific processes. Current understanding of the role of mitochondria in both the innate and adaptive immune systems is expanding. Concurrently, immunodeficiencies arising from perturbation of mitochondrial elements are increasingly recognized. Recent observations of immune dysfunction and increased incidence of infection in patients with primary mitochondrial disorders further support an important role for mitochondria in the proper function of the immune system. Here we review current findings.