Homogentisic Acid

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Related to Homogentisate: PKU

homogentisic acid

[¦hä·mə‚jen¦tiz·ik ′as·əd]
(biochemistry)
C8H8O4 An intermediate product in the metabolism of phenylalanine and tyrosine; found in excess in persons with phenylketonuria and alkaptonuria.

Homogentisic Acid

 

an intermediate product of the decomposition of the cyclic amino acids phenylalanine and tyrosine in the animal or human body. In the hereditary disease alkaptonuria, which is expressed in a disturbance of the metabolism of these amino acids, the process stops at the stage of homogentisic acid formation because of an absence of homogentisic acid oxidase, and homogentisic acid is discharged with the urine.

References in periodicals archive ?
Defective production of homogentisate 1,2-dioxygenase results in the accumulation of HGA, a tyrosine degradation product, in the bloodstream.
Reduction of tyrosine and phenylalanine in the diet has been reported to reduce the excretion of homogentisate.
1) Caused by mutations in the homogentisate 1, 2-dioxygenase gene, (2) it occurs in less than 1 in a million births.