Enzyme deficient in alkaptonuria is
Ans. a (Homogentisic acid oxidase). (Ref. Harper, Biochemistry, 25th ed., 332)Disorders of amino acid and urea cycle metabolismAmino acid metabolism NameEnzyme deficiencyMetabolism affected1AlbinismTyrosinaseTyrosine2AlkaptonuriaHomogentisate oxidasePhenylalanine, tyrosine3CystathioninuriaCystathionine lyaseCysteine, methionine, serine4HistidinemiaHistidine ammonia lyaseHistidine5HomocystinuriaCysthionine sythaseCysteine, methionine, serine6Maple syrup urine diseaseBranched-chain keto acid dehydrogenaseValine, isoleucine, leucine7Phenylketonuria(PKU)Phenylanine hydroxylasePhenylalanine8TyrosinosisHydroxyphenylpyruvate hydroxylaseTyrosine transaminase Urea cycle1ArgininemiaArginase2ArgininosuccinaturiaArgininosuccinate lyase3CitrullinemiaArgininosuccinate synthetase4Hyperammonemia-IOrnithine transcarbamoylase5Hyperammonemia-llCarbamoyl-phosphate synthetase-l
Homogentisic acid oxidase
Tyrosinase I
Tyrosinase II
Acid maltase
Enzymes