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homogentisate 1,2-dioxygenase An iron-containing enzyme that catalyses the oxidative cleavage of the benzene ring in homogentisic acid by O2, forming 4-maleylacetoacetate; an absence or deficiency of this enzyme will result in alcaptonuria.
Synonym: homogentisic acid oxidase.
(05 Mar 2000)
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  • Homogentisate 1,2-Dioxygenase - »õâ A mononuclear Fe(II)-dependent oxygenase, this enzyme catalyzes the conversion of homogentisate to 4-maleylacetoacetate, the third step in the pathway for the catabolism of TYROSINE. Deficiency in the enzyme causes ALKAPTONURIA, an autosomal recessive disorder, characterized by homogentisic aciduria, OCHRONOSIS and ARTHRITIS. This enzyme was formerly characterized as EC 1.13.1.5 and EC 1.99.2.5.
    Synonyms : Homogentisate Dioxygenase, Homogentisate Oxidase, Homogentisate Oxygenase, Homogentisic Acid Oxidase, 1, 2-Dioxygenase, Homogentisate, Acid Oxidase, Homogentisic, Dioxygenase, Homogentisate, Homogentisate 1, 2 Dioxygenase, Oxidase, Homogentisate
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homogentisate the anionic form of homogentisic acid.
Ãâó: www.mercksource.com/pp/us/cns/cns_health_library.j...
homogentisate 1,2-dioxygenase [EC 1.13.11.5] an enzyme of the oxidoreductase class that catalyzes the oxidation of homogentisate to form 4-methylacetoacetate as a step in the degradation of tyrosine and phenylalanine. Deficiency of the enzyme, an autosomal recessive trait, causes alkaptonuria.
Ãâó: www.mercksource.com/pp/us/cns/cns_health_library.j...
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