Deficiency of which of the following enzymes is associated with Tyrosinosis:
Correct Answer: Fumarylacetoacetate hydroxylase
Description: Tyrosinosis is synonymous with Type-1 Tyrosinemia and is caused by a deficiency of Enzyme Fumarylacetoacetate hydrolase. Tyrosine Catabolic pathway and Associated Disorders: Tyrosine aminotransferase 4-Hydroxyphenylpyruvate dioxygenase Homogentisate oxidase Fumarylacetoacetatehydrolase Tyrosine aminotransferase Tyrosinemia type II (Richner-Hanha sydrome) 4-Hydroxyphenylpyruvate deoxygenase Tyrosinemia type III (Complete deficiency of Enzyme) 4-Hydroxyphenylpyruvate dioxygenase complex Transient Tyrosinemia of the new-born (immaturity of Enzyme ) 4-Hydroxyphenylpyruvate dioxygenase complex Hawkinsinuria (Mutant enzyme) Catalyses a paial reaction Fumarylacetoacetate hydrolase Tyrosinemia type I (Tyrosinosis) Homogentisate oxidase Alkaptonuria Disorders associated with 4-Hydroxyphenylpyruvate dioxygenase: Immaturity of 4-hydroxyphenylpyruvate dioxygenase (4HPPD) enzyme Transient neonatal Tyrosinemia (TNT) is a form of hypeyrosinemia produced by the complete deficiency of 4-Hydroxyphenylpyruavate dioxygenase (4HPPD) leads to Tyrosinemia III. Ref: Harper 30th edition Pgno: 304
Category:
Biochemistry
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