Defective fumarylacetoacetate hydrolase enzyme is associated with
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