Reactome | p-hydroxyphenylpyruvate + O2 => homogentisate + CO2

p-hydroxyphenylpyruvate + O2 => homogentisate + CO2

Stable Identifier

R-HSA-71163

Type

Reaction [transition]

Species

Homo sapiens

Compartment

cytosol

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p-hydroxyphenylpyruvate + O2 => homogentisate + CO2

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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 Unpublished crystallographic data indicate that the enzyme is a homodimer (PDB 3ISQ) utilising Fe2+ and ascorbate (AscH-).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
10942115	Mutations in the 4-hydroxyphenylpyruvate dioxygenase gene (HPD) in patients with tyrosinemia type III Rüetschi, U, Cerone, R, Pérez-Cerda, C, Schiaffino, MC, Standing, S, Ugarte, M, Holme, E	Hum Genet	2000
5487549	The mechanism of enzymic formation of homogentisate from p-hydroxyphenylpyruvate Lindblad, B, Lindstedt, G, Lindstedt, S	J. Am. Chem. Soc.	1970