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Epub 2009 Jun 25

About 10% of molecular oxygen undergoes ROS-yielding stepwise reduction during such physiological processes as mitochondrial respiration, CYP-mediated processing of endogenous lipophilic compounds, phagocytosis, and synthesis of disulphide (S-S) bonds from cysteine that are needed for proper protein folding in the endoplasmic reticulum (ER) (143)

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This article provides a comprehensive summary of the pathogenesis, clinical features, diagnosis and treatment of epilepsy associated with organic acid metabolism disorders