Nutritional Interventions for Mitochondrial OXPHOS Deficiencies: Mechanisms and Model Systems.

ABSTRACT: Multisystem metabolic disorders caused by defects in oxidative phosphorylation (OXPHOS) are severe, often lethal, conditions. Inborn errors of OXPHOS function are termed primary mitochondrial disorders (PMDs), and the use of nutritional interventions is routine in their supportive management. However, detailed mechanistic understanding and evidence for efficacy and safety of these interventions are limited. Preclinical cellular and animal model systems are important tools to investigate PMD metabolic mechanisms and therapeutic strategies. This review assesses the mechanistic rationale and experimental evidence for nutritional interventions commonly used in PMDs, including micronutrients, metabolic agents, signaling modifiers, and dietary regulation, while highlighting important knowledge g

SUBMITTER: Kuszak AJ 

PROVIDER: S-EPMC5911915 | biostudies-literature | 2018 Jan

REPOSITORIES: biostudies-literature