This is a real-world medical entrance exam question, please give the true answer based on the question and selection. Topic name: Disorders of Amino acid Metabolism, Subject name: Pediatrics
[{'content': "Amino acid metabolism is implicated in which disease? \nSelect and explain. \nA. Maple syrup urine disease, B. Reye's syndrome, C. Von Gierke's disease, D. McArdle's diseases", 'role': 'user'}, {'content': "Answer: A \nExplanation: OPTION 1: Maple syrup urine disease - It is inborn error of metabolism of branched chain amino acids Von Gierke's disease and McArdle's diseases are glycogen storage diseases. Reye's syndrome is an acute self - limiting metabolic insult resulting in generalized mitochondrial dysfunction due to inhibition of fatty acid oxidation.", 'role': 'assistant'}]