This is a real-world medical entrance exam question, please give the true answer based on the question and selection. Topic name: Metabolism of protein and amino acid, Subject name: Biochemistry
[{'content': 'The following enzyme defect causes acute intermittent porphyria \nSelect and explain. \nA. Uroporphyrinogen III synthase, B. Uroporphyrinogen decarboxylase, C. Hydroxymethylbilane synthase, D. Protoporphyrinogen oxidase', 'role': 'user'}, {'content': 'Answer: C \nExplanation: The defective enzyme in acute intermittent porphyria is hydroxymethylbilane synthase (uroporphyrinogen I synthase)ALA and porphyrobilinogen accumulate in body tissues and fluids.It is an autosomal dominant disorder.While most porphyrias are inherited in an autosomal dominant manner, congenital erythropoietic porphyria is inherited in a recessive mode. The defective enzyme in congenital erythropoietic porphyria is uroporphyrinogen III synthase,Harper 30th edition pg: 329', 'role': 'assistant'}]