Reactome | homogentisate + O2 => maleylacetoacetate

homogentisate + O2 => maleylacetoacetate

Stable Identifier

R-HSA-71164

Type

Reaction

Species

Homo sapiens

Compartment

cytosol

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homogentisate + O2 => maleylacetoacetate

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Cytosolic homogentisate 1,2-dioxygenase (HGD) catalyzes the reaction of homogentisate and molecular oxygen to form 4-maleylacetoacetate. The activity of human HGD is inferred from the phenotype of alkaptonuria patients, in whom HGD activity is defective (Fernandez-Canon et al. 1996). HGD is a homohexamer (Titus et al. 2000).

Literature References

PubMed ID	Title	Journal	Year
10876237	Crystal structure of human homogentisate dioxygenase. Timm, DE, Burgner, J, Titus, GP, Mueller, HA, Rodriguez De Córdoba, S, Peñalva, MA	Nat Struct Biol	2000
8782815	The molecular basis of alkaptonuria Fernández-Ruiz, E, Fernández-Cañón, JM, Granadino, B, Renedo, M, Beltrán-Valero de Bernabé, D, Rodriguez De Córdoba, S, Peñalva, MA	Nat Genet	1996