homogentisic acid

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Related to Homogentisate: PKU

homogentisic acid

 [ho″mo-jen-tis´ik]
2,5-dihydroxyphenyl acetic acid, an intermediate product in the metabolism of tyrosine and phenylalanine, excreted in the urine in the inborn error of metabolism known as phenylketonuria.
Miller-Keane Encyclopedia and Dictionary of Medicine, Nursing, and Allied Health, Seventh Edition. © 2003 by Saunders, an imprint of Elsevier, Inc. All rights reserved.

ho·mo·gen·tis·ic ac·id

(hō'mō-jen-tis'ik as'id),
Glycosuric acid; (2,5-dihydroxyphenyl)acetic acid; an intermediate in l-phenylalanine and l-tyrosine catabolism; if made alkaline, it oxidizes rapidly in air to a quinone that polymerizes to a melaninlike material; elevated levels are observed in patients with alcaptonuria.
Synonym(s): alcapton, alkapton
Farlex Partner Medical Dictionary © Farlex 2012

homogentisic acid

(hō′mō-jĕn-tĭz′ĭk)
n.
An intermediate of the metabolic breakdown of tyrosine and phenylalanine; it occurs in the urine in cases of alkaptonuria.
The American Heritage® Medical Dictionary Copyright © 2007, 2004 by Houghton Mifflin Company. Published by Houghton Mifflin Company. All rights reserved.

ho·mo·gen·tis·ic ac·id

(hō'mō-jen-tis'ik as'id)
An intermediate in l-phenylalanine and l-tyrosine catabolism; if made alkaline, it oxidizes rapidly in air to a quinone that polymerizes to a melaninlike material; elevated levels are observed in individuals having alcaptonuria.
Synonym(s): alcapton, alkapton.
Medical Dictionary for the Health Professions and Nursing © Farlex 2012

homogentisic acid

see GARROD, A.
Collins Dictionary of Biology, 3rd ed. © W. G. Hale, V. A. Saunders, J. P. Margham 2005
References in periodicals archive ?
Reduction of tyrosine and phenylalanine in the diet has been reported to reduce the excretion of homogentisate. It is not known whether dietary restriction from early life would avoid or minimise later complications, but such an approach is reasonable.
Ochronosis is a musculoskeletal manifestation of alkaptonuria, a rare disease caused by a loss-of-function mutation on chromosome 3q, which leads to a defect in the HGO, or homogentisate 1, 2-dioxygenase enzyme.
When a patient has such a mutation, he or she manufactures a defective version of the protein homogentisate dioxygenase and, thus, suffers from alkaptonuria.
Homogentisate 1,2 dioxygenase is expressed in human osteoarticular cells: implications in alkaptonuria.
Alkaptonuria (AKU; MIM number 203500) is an ultra-rare (1: 250.000-1.000.000 incidence) autosomal recessive inborn error of catabolism of the aromatic amino acids due to a deficient activity of the enzyme homogentisate 1,2-dioxygenase (HGD) leading to the accumulation of homogentisic acid (2,5-dihydroxyphenylacetic acid; HGA).