Oxidation of homogentisic acid to ochronotic pigment in connective tissue

Add time:07/26/2019    Source:sciencedirect.com

Ochronotic pigmentation of connective tissues occurs in individuals with alcaptonuria. An enzyme system, termed homogentisic acid polyphenol oxidase, has been identified in mammalian skin and cartilage which catalyzes the oxidation of homogentisic acid to an “ochronotic-like” pigment. Benzoquinoneacetic acid, a probable intermediary compound in the oxidation and polymerization reactions also serves as a substrate for the oxidase. Benzoquinoneacetic acid has been identified as an intermediary metabolite in the oxidation of homogentisic acid to the pigmented polymerized product. Studies with partially purified enzyme from albino guinea-pig skin indicate that copper is essential for enzymatic activity. A pathway is proposed for the oxidation of homogentisic acid via benzoquinoneacetic acid to ochronotic pigment in the tissues of alcaptonuric individuals.

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