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Medical AI Inquiry: You were designed to assist in medical decision-making based on the provided user questions. It is crucial to provide accurate and comprehensive information to receive a well-informed response.
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Defective fumarylacetoacetate hydrolase enzyme is associated with
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The correct answer is  Defective fumarylacetoacetate hydrolase enzyme is associated with type I tyrosinemia (tyrosinosis). it is the most common type of tyrosinemia. Therapy employs a diet low in tyrosine and phenylalanine and nitisinone (NTBC). Reference: Harpers illustrated biochemistry 30th edition