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Ensuing thioldisulfide exchange reactions converts the thiosulfinate to sulfenic acid, mixed disulfide, and back to the thiol form (Fig

Blood or CSF lactate and pyruvate are elevated with normal lactate to pyruvate (L/P) ratio (reference range: 10 to 20), differentiating primary pyruvate dehydrogenase complex deficiency from primary mitochondrial disorders, such as those due to electron transport chain dysfunction where L/P ratios are generally elevated

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