homogentisate + O2 => maleylacetoacetate

Stable Identifier
R-HSA-71164
Type
Reaction
Species
Homo sapiens
Compartment
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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
Participants
Participates
Catalyst Activity

homogentisate 1,2-dioxygenase activity of HGD hexamer [cytosol]

Orthologous Events
Cross References
Rhea
Authored
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