Homogentisate 1,2-dioxygenase (IPR005708)

Short name: Homogentis_dOase

Overlapping homologous superfamilies

Family relationships


Alkaptonuria (AKU), a rare hereditary disorder, was the first disease to be interpreted as an inborn error of metabolism. The deficiency causes homogentisic aciduria, ochronosis, and arthritis. AKU patients are deficient for homogentisate 1,2 dioxygenase (EC:, the enzyme that mediates the conversion of homogentisate to maleylacetoacetate; a step in the catabolism of both tyrosine and phenylalanine. Homogentisate + O(2) = 4-maleylacetoacetate.

GO terms

Biological Process

GO:0006559 L-phenylalanine catabolic process
GO:0055114 oxidation-reduction process
GO:0006570 tyrosine metabolic process

Molecular Function

GO:0004411 homogentisate 1,2-dioxygenase activity

Cellular Component

No terms assigned in this category.

Contributing signatures

Signatures from InterPro member databases are used to construct an entry.