Enzyme deficient in tyrosinemia type 1 ?
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.
Phenylalanine hydroxylase
Tyrosinase
Fumarylacetoacetate hydroxylase
Tyrosine transaminase