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).
Timm, DE, Burgner, J, Titus, GP, Mueller, HA, Rodriguez De Córdoba, S, Peñalva, MA
Fernández-Ruiz, E, Fernández-Cañón, JM, Granadino, B, Renedo, M, Beltrán-Valero de Bernabé, D, Rodriguez De Córdoba, S, Peñalva, MA
homogentisate 1,2-dioxygenase activity of HGD hexamer [cytosol]
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