p-OH phenyl pyruvate hydroxylase (4-hydroxyphenylpyruvate dioxygenase, 4HPPD, HPD, HPPDase, 4-hydroxyphenylpyruvic acid oxidase, HPD, PPD)

Function

• amino-acid degradation, L-phenylalanine degradation
• acetoacetate & fumarate from L-phenylalanine, step 3/6

4-hydroxyphenylpyruvate + O2 <--> homogentisate + CO2

Cofactor: binds 1 Fe+2/Fe+3 per subunit (putative)

Structure

• homodimer
• belongs to the 4HPPD family

Pathology

• defects in HPD are the cause of tyrosinemia type 3
• defects in HPD are a cause of hawkinsinuria

More general terms

• dioxygenase
• hydroxylase; monooxygenase
• phosphoprotein

Additional terms

• 4-hydroxyphenylpyruvate dioxygenase-like protein (glyoxalase domain-containing protein 1, HPDL, GLOXD1)

Database

• OMIM: https://mirror.omim.org/entry/140350
• OMIM: https://mirror.omim.org/entry/276710
• UniProt: http://www.uniprot.org/uniprot/P32754.html