Centre de référence Maladies Métaboliques, Hôpital Robert Debré, APHP, Université Paris 7, Faculté de médecine Denis Diderot, IFR02, INSERM, U676, Paris, France.
Nutr Rev. 2011 Feb;69(2):65-75. doi: 10.1111/j.1753-4887.2010.00363.x. Epub 2011 Jan 14.
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.
线粒体发挥着至关重要的生理功能，在营养、健康和疾病之间建立了复杂的联系。虽然线粒体功能障碍导致氧化磷酸化（OXPHOS）受损是罕见遗传性 OXPHOS 疾病的标志，但 OXPHOS 功能障碍也在 2 型糖尿病和各种神经退行性疾病等常见疾病的病理生理学中发挥核心作用。饮食干预，特别是热量限制，已被证明可以改善这些疾病的进程并延长寿命。关于营养保健品（宏量营养素、维生素和辅因子）对原发性遗传性 OXPHOS 疾病的影响的数据很少。这篇综述介绍了营养调节对线粒体和寿命调节的影响以及潜在的线粒体功能障碍疾病治疗方法的最新进展。
