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

@article{Kuszak2018NutritionalIF,
  title={Nutritional Interventions for Mitochondrial OXPHOS Deficiencies: Mechanisms and Model Systems.},
  author={Adam J. Kuszak and Michael Graham Espey and Marni J. Falk and Marissa A. Holmbeck and Giovanni Manfredi and Gerald S Shadel and Hilary J Vernon and Zarazuela Zolkipli-Cunningham},
  journal={Annual review of pathology},
  year={2018},
  volume={13},
  pages={163-191}
}
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… CONTINUE READING
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