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Decreased Binding of 14C-Homogentisic Acid induced by Ascorbic Acid in Connective Tissues of Rats with Experimental Alcaptonuria

Nature volume 228, pages 770–771 (1970)Cite this article

Abstract

ADULTS with alcaptonuria develop a destructive arthritis secondary to the deposition of a melanin-like pigment in their connective tissues, especially skin, tendon and Cartilage (ochronosis). The biochemical steps leading to ochronosis involve the chemical binding of oxidized and/or polymerized derivatives of homogentisic acid (HGA), the metabolite accumulating in the blood in alcaptonuria, to these connective tissues, with subsequent physico-chemical alterations in the properties of collagen1. HGA seems to be converted to an oxidized polymerized derivative through the formation of a labile, oxidized intermediate, benzoquinone acetic acid (BQA). Both steps in this conversion are catalysed by HGA polyphenol oxidase, a copper-requiring enzyme2.

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Author notes

  1. THOMAS J. LUSTBERG

    Present address: Vanderbilt University School of Medicine, Nashville, Tennessee

  2. J. EDWIN SEEGMILLER

    Present address: Department of Medicine, University of California, San Diego, La Jolla, California

Authors and Affiliations

  1. Section on Human Biochemical Genetics, National Institute of Arthritis and Metabolic Diseases, National Institutes of Health, Bethesda, Maryland

    THOMAS J. LUSTBERG, JOSEPH D. SCHULMAN & J. EDWIN SEEGMILLER

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  1. THOMAS J. LUSTBERG
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  2. JOSEPH D. SCHULMAN
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  3. J. EDWIN SEEGMILLER
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LUSTBERG, T., SCHULMAN, J. & SEEGMILLER, J. Decreased Binding of 14C-Homogentisic Acid induced by Ascorbic Acid in Connective Tissues of Rats with Experimental Alcaptonuria. Nature 228, 770–771 (1970). https://doi.org/10.1038/228770a0

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