Reactome | homogentisate + O2 => maleylacetoacetate

homogentisate + O2 => maleylacetoacetate

Stable Identifier

R-HSA-71164

Type

Reaction [transition]

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