Which enzyme deficiency causes tyrosinemia type 1?
Fumarylacetoacetate is then hydrolyzed to fumarate and acetoacetate by a hydrolase (step 6, Fig.17.2). This results in the production of a glucogenic product (fumarate) and a ketone body (acetoacetate). Hence phenylalanine and tyrosine are paly glucogenic and paly ketogenic. Impoant Specialized Products from TyrosineRef: DM Vasudevan - Textbook of Biochemistry, 6th edition, page no: 204
Fumaryl acetoacetate hydrolase
Tyrosine transaminase
HGA oxidase
Tyrosinase
vitamins