p-hydroxyphenylpyruvate + O2 => homogentisate + CO2

Stable Identifier
R-HSA-71163
Type
Reaction
Species
Homo sapiens
Compartment
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Cytosolic 4-hydroxyphenylpyruvate dioxygenase (HPD) catalyzes the reaction of 3-(4-Hydroxyphenyl)pyruvate with molecular oxygen to form homogentisate and CO2 (Ruetschi et al. 2000, Lindblad et al. 1970). Unpublished crystallographic data indicate that the enzyme is a homodimer (PDB 3ISQ) utilising Fe2+ and ascorbate (AscH-).

Literature References
PubMed ID Title Journal Year
5487549 The mechanism of enzymic formation of homogentisate from p-hydroxyphenylpyruvate

Lindblad, B, Lindstedt, S, Lindstedt, G

J. Am. Chem. Soc. 1970
10942115 Mutations in the 4-hydroxyphenylpyruvate dioxygenase gene (HPD) in patients with tyrosinemia type III

Schiaffino, MC, Standing, S, Pérez-Cerda, C, Rüetschi, U, Holme, E, Ugarte, M, Cerone, R

Hum Genet 2000
Participants
Participates
Catalyst Activity

4-hydroxyphenylpyruvate dioxygenase activity of HPD:AscH-:Fe2+ dimer [cytosol]

Orthologous Events
Cross References
Rhea
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