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What is Defective fumarylacetoacetate hydrolase enzyme is associated with and explain in detail?
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