Reactome | HGD dioxygenates homogentisate

HGD dioxygenates homogentisate

Stable Identifier

R-HSA-71164

Type

Reaction [transition]

Species

Homo sapiens

Compartment

cytosol

Synonyms

homogentisate + O2 => maleylacetoacetate

ReviewStatus

5/5

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Cytosolic homogentisate 1,2-dioxygenase (HGD) catalyzes the reaction of homogentisate and molecular oxygen to form 4-maleylacetoacetate. HGD is a homohexamer, with each monomer binding one Fe(2+) ion as a catalytic cofactor (Titus et al., 2000; Veldhuizen et al., 2005). HGD activity is defective in alkaptonuria patients who excrete excess homogentisate and its oxidized form alkapton in the urine, with further metabolic complications (MIM:203500; Fernandez-Canon et al. 1996; reviewed in Zatkova et al., 2020).

Literature References

PubMed ID	Title	Journal	Year
15479158	Steady-state kinetics and inhibition of anaerobically purified human homogentisate 1,2-dioxygenase Tanguay, RM, Whiting, CJ, Gingras, G, Vaillancourt, FH, Boukouvalas, J, Veldhuizen, EJ, Eltis, LD, Xiao, Y, Hsiao, MM	Biochem J	2005
32158253	Alkaptonuria: Current Perspectives Ranganath, L, Kadasi, L, Zatkova, A	Appl Clin Genet	2020
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