homogentisic acid

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Related to Homogentisate: PKU
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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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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.
AKU is caused by the deficiency of the homogentisate 1,2-dioxygenase enzyme which results in excretion of large quantities of homogentisic acid (HGA) in the urine and a slowly progressive deposition of HGA and its oxidative product in cartilage, intervertebral disk and other connective tissues, leading to ochronotic arthropathy.
This enzyme helps break down homogentisate in 50 maleyil acetoacetate.