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).
Fernández-Cañón, JM, Granadino, B, Beltrán-Valero de Bernabé, D, Renedo, M, Fernández-Ruiz, E, Peñalva, MA, Rodriguez De Córdoba, S
Titus, GP, Mueller, HA, Burgner, J, Rodriguez De Córdoba, S, Peñalva, MA, Timm, DE
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