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': 'HHH syndrome is caused by defect in: \nSelect and explain. \nA. Ornithine permease, B. Ornithine transcarbamyolase, C. Arginase, D. Argininosuccinase lyase', 'role': 'user'}, {'content': 'Answer: A \nExplanation: Hyperammonemia-Hyperornithinemia-Homocitrullinemia (HHH) Syndrome: autosomal recessive disorder Defect is in transpo system of ornithine from cytosol into mitochondria Accumulation of ornithine in the cytosol and a deficiency of ornithine inside mitochondria.', 'role': 'assistant'}]