homogentisic acid

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Noun1.homogentisic acid - an acid formed as an intermediate product of the metabolism of tyrosine and phenylalanine
acid - any of various water-soluble compounds having a sour taste and capable of turning litmus red and reacting with a base to form a salt
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Errors in amino acid metabolism conclude some correlations between biochemical and pathological conditions, for example, alkaptonuria, an inherited metabolic disorder is caused by the absence of enzyme homogentisate oxidase due to which accumulation of homogentisate occurs and is excreted in urine, which turns dark black on standing due to oxidation.
The 3-hydroxykynurenine, homogentisate and tyrosine biomarkers were identified before dementia, and 1methylnicotinamide, 2-oxoglutarate, citrate, urea, dimethylamine, trigonelline and trimethylamine (42) could be identified in the advanced stages of the disease.
The deficiency of homogentisate 1,2 dioxygenate (HGD) leads to an accumulation of homogentisic acid (HGA) in plasma and urine which auto-oxidizes in tissues into benzoquinone acetic acid and polymerizes to an ochronotic pigment.
Alkaptonuria is a rare inborn error of metabolism caused by mutations in the gene responsible for the production of homogentisate 1,2-dioxygenase, an enzyme that plays an important role in the normal degradation of the aromatic amino acids tyrosine and phenylalanine.
This patient has alkaptonuria, a rare disorder (approximately 1 in 250 000) caused by a deficiency of the enzyme homogentisate 1,2-dioxygenase ([HGD.sup.3]; EC.1.13.11.5).