title: Mitochondrial response to controlled nutrition in health and disease.
abstract: Mitochondria exert crucial physiological functions that create complex links among nutrition, health, and disease. While mitochondrial dysfunction with subsequent impairment of oxidative phosphorylation (OXPHOS) is the hallmark of the rare inherited OXPHOS diseases, OXPHOS dysfunction also plays a central role in the pathophysiology of common conditions such as type 2 diabetes and various neurodegenerative disorders. Dietary interventions, especially calorie restriction, have been shown to improve the course of these diseases and to extend the lifespan. Few data are available on the impact of nutraceuticals (macronutrients, vitamins, and cofactors) on primary inherited OXPHOS diseases. This review presents recent knowledge about the impact of nutritional modulation on mitochondria and lifespan regulation and about the development of potential treatments for mitochondrial dysfunction diseases.
authors.affiliation: Centre de référence Maladies Métaboliques, Hôpital Robert Debré, APHP, Université Paris 7, Faculté de médecine Denis Diderot, IFR02, INSERM, U676, Paris, France. manuel.schiff@rdb.aphp.fr
authors.forename: Manuel
authors.initials: M
authors.lastname: Schiff
authors.forename: Paule
authors.initials: P
authors.lastname: Bénit
authors.forename: Assetou
authors.initials: A
authors.lastname: Coulibaly
authors.forename: Sandrine
authors.initials: S
authors.lastname: Loublier
authors.forename: Riyad
authors.initials: R
authors.lastname: El-Khoury
authors.forename: Pierre
authors.initials: P
authors.lastname: Rustin
mesh_terms: D000375:Aging / Q000502:physiology; D031204:Caloric Restriction; D003924:Diabetes Mellitus, Type 2 / Q000178:diet therapy / Q000378:metabolism; D055423:Diet, Ketogenic; D004734:Energy Metabolism / Q000502:physiology; D006801:Humans; D008928:Mitochondria / Q000378:metabolism / Q000502:physiology*; D028361:Mitochondrial Diseases / Q000178:diet therapy* / Q000378:metabolism* / Q000503:physiopathology; D019636:Neurodegenerative Diseases / Q000178:diet therapy / Q000378:metabolism; D010085:Oxidative Phosphorylation