Cytosolic homogentisate 1,2-dioxygenase (HGD) catalyzes the ...

created [InstanceEdit:179407] Gopinathrao, G, 2006-04-27 13:13:48
dbId 178870
displayName Cytosolic homogentisate 1,2-dioxygenase (HGD) catalyzes the ...
modified [InstanceEdit:9855783] Stephan, Ralf, 2023-12-07
schemaClass Summation
text 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).