Homogentisic acid enzyme is an enzyme that is responsible for breaking down homogentisic acid, an intermediary in the metabolism of tyrosine. Homogentisic acid is a toxic intermediate in the production of melanin, a pigment found in the skin, eyes, and hair of humans and other animals. Homogentisic acid is produced when the amino acid tyrosine is broken down by the enzyme tyrosinase. If the enzyme homogentisic acid enzyme is deficient, the homogentisic acid accumulates in the body and can lead to a condition known as alkaptonuria. Alkaptonuria is a rare, inherited disorder characterized by the accumulation of homogentisic acid in the body tissues, which can lead to joint pain, dark urine, and kidney stones.