Enzyme deficient in tyrosinemia type 1 ?: (A) Phenylalanine hydroxylase, (B) Tyrosinase, (C) Fumarylacetoacetate hydroxylase, (D) Tyrosine transaminase
Answer is C. TyrosinemiaIt is a defect in metabolism of tyrosine. It may be of following types :-Tyrosinemia type-I (tyrosinosis/hepatorenal syndrome) :- It is due to defect in fumarylacetoacetate hydroxylase deficiency. Patients with chronic tyrosinosis are prone to develop cirrhosis and hepatic carcinoma. There is cabbage like odor in acute tyrosinosis.Tyrosinemia type - II (Richer-Hanha syndrome) :- It is due to deficiency of tyrosine transaminase (tyrosine aminotrans-ferase).Neonatal tyrosinemia : - It is due to deficiency of hydroxyphenyl pyruvate hydroxylase.