title: Powering the immune system: mitochondria in immune function and deficiency.
abstract: 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.
authors.affiliation: Department of Neurology, Massachusetts General Hospital, Boston, MA 02114, USA.
authors.forename: Melissa A
authors.initials: MA
authors.lastname: Walker
authors.affiliation: Department of Neuroscience, Rehabilitation, Ophthalmology, Genetics, Maternal and Child Health (DINOGMI), University of Genoa, Genoa, 16147 Genova, Italy.
authors.forename: Stefano
authors.identifier: 0000-0002-7129-868X
authors.initials: S
authors.lastname: Volpi
authors.affiliation: Department of Neurology, Massachusetts General Hospital, Boston, MA 02114, USA.
authors.forename: Katherine B
authors.initials: KB
authors.lastname: Sims
authors.affiliation: Division of Allergy and Immunology, Department of Pediatrics, Massachusetts General Hospital for Children, Boston, MA 02114, USA.
authors.forename: Jolan E
authors.initials: JE
authors.lastname: Walter
authors.affiliation: Novartis Institute for Research in Biomedicine, Basel, Switzerland.
authors.forename: Elisabetta
authors.initials: E
authors.lastname: Traggiai
mesh_terms: D056704:Adaptive Immunity / Q000276:immunology*; D020022:Genetic Predisposition to Disease / Q000235:genetics; D006801:Humans; D007107:Immune System / Q000166:cytology / Q000276:immunology* / Q000378:metabolism; D007113:Immunity, Innate / Q000276:immunology*; D008928:Mitochondria / Q000235:genetics / Q000276:immunology* / Q000378:metabolism; D028361:Mitochondrial Diseases / Q000235:genetics / Q000276:immunology* / Q000378:metabolism; D018448:Models, Immunological; D009154:Mutation