4-Hydroxyphenylpyruvate dioxygenase

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4-hydroxyphenylpyruvate dioxygenase
Identifiers
Symbol	HPD
Alt. Symbols	PPD;
Entrez	3242
HUGO	5147
OMIM	609695
RefSeq	NM_002150
UniProt	P32754
Other data
EC number	1.13.11.27
Locus	Chr. 12 q24-qter

4-Hydroxyphenylpyruvate dioxygenase HPD is a Cu-containing enzyme, that catalyzes the third reaction in the catabolism of phenylalanine and tyrosine - the conversion of 4-hydroxyphenylpyruvate to homogentisate.

Function

The HPD reaction occurs through a NIH shift and involves the oxidative decarboxylation of an α-oxo acid as well as aromatic ring hydroxylation. The NIH-shift, which has been demonstrated through isotope-labeling studies, involves migration of an alkyl group to form a more stable carbocation. The shift, accounts for the observation that C3 is bonded to C4 in 4-hydroxyphenylpyruvate but to C5 in homogentisate.

See also

• 4-Alpha-hydroxyphenylpyruvate hydroxylase deficiency

v • d • e Oxidoreductases: monooxygenases (EC 1.13)
1.13.11 - two atoms of oxygen	Catechol dioxygenase - Homogentisate 1,2-dioxygenase - Cysteine dioxygenase - 4-Hydroxyphenylpyruvate dioxygenase - Indoleamine 2,3-dioxygenase - Lipoxygenase (5)
1.13.12 - one atom of oxygen	Firefly luciferase
1.13.99 - other	Inositol oxygenase

v • d • e Amino acid metabolism enzymes (essential in CAPS)
K	LYSINE→acetyl-CoA Saccharopine dehydrogenase - Glutaryl-CoA dehydrogenase LEUCINE→acetyl-CoA Branched-chain alpha-keto acid dehydrogenase complex - Isovaleryl coenzyme A dehydrogenase - Methylcrotonyl-CoA carboxylase TRYPTOPHAN→acetyl-CoA Kynureninase PHENYLALANINE→tyrosine→acetoacetate (see below)
G	glycine→serine→pyruvate→TCA Serine hydroxymethyltransferase - Serine dehydratase alanine→pyruvate→TCA Alanine transaminase cysteine→pyruvate→TCA D-cysteine desulfhydrase threonine→pyruvate→TCA L-threonine dehydrogenase HISTIDINE→glutamate Histidine ammonia-lyase - Urocanate hydratase - Formiminotransferase cyclodeaminase proline→glutamate Proline oxidase - Pyrroline-5-carboxylate reductase - 1-Pyrroline-5-carboxylate dehydrogenase/ALDH4A1 - PYCR1 arginine→glutamate Ornithine aminotransferase - Ornithine decarboxylase - Agmatinase glutamate→alpha-ketoglutarate→TCA Glutamate dehydrogenase METHIONINE→propionyl-CoA Methionine adenosyltransferase - Adenosylhomocysteinase - (or MTR/Homocysteine methyltransferase) - Cystathionine beta synthase - Cystathionine gamma-lyase ISOLEUCINE/valine→propionyl-CoA Branched-chain alpha-keto acid dehydrogenase complex
NT	HISTIDINE→histamine Histidine decarboxylase tyrosine→dopamine→epinephrine Tyrosine hydroxylase - Aromatic L-amino acid decarboxylase - Dopamine beta hydroxylase - Phenylethanolamine N-methyltransferase - Catechol-O-methyl transferase glutamate→GABA Glutamate decarboxylase arginine→NO Nitric oxide synthase
Other	tyrosine→melanin Tyrosinase TRYPTOPHAN→serotonin→melatonin Tryptophan hydroxylase - Aromatic L-amino acid decarboxylase - Acetylserotonin O-methyltransferase cysteine+glutamate→glutathione Gamma-glutamylcysteine synthetase - Glutathione synthetase glycine→creatine Guanidinoacetate N-methyltransferase - Creatine kinase glutamate→glutamine Glutamine synthetase - Glutaminase collagen Prolyl hydroxylase - Lysyl hydroxylase
see also disorders, intermediates

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