Prolyl hydroxylase

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Prolyl hydroxylase (or procollagen-proline dioxygenase) is an enzyme involved in the production of collagen, acting to hydroxylate proline to hydroxyproline.

It requires vitamin C as a cofactor.

Reaction: Proline + α-ketoglutarate + O₂ + Fe²⁺ → 4-hydroxyproline + Fe⁴⁺ + CO₂ + succinate

The process requires ascorbic acid which provides Fe²⁺as a reductant to provide two electrons.

It is classified under EC 1.14.11.2.

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• MeSH Prolyl+hydroxylase

v • d • e Oxidoreductases: dioxygenases, including steroid hydroxylases (EC 1.14) 1.14.11 - 2-oxoglutarate Prolyl hydroxylase - Lysyl hydroxylase 1.14.13 - NADH or NADPH Flavin-containing monooxygenase - Nitric oxide synthase - Cholesterol 7 alpha-hydroxylase - Methane monooxygenase - 3A4 -51A1 1.14.14 - reduced flavin or flavoprotein 19A1 - 2D6 - 2E1 1.14.15 - reduced iron-sulfur protein 11B1 - 11B2 - 11A1 1.14.16 - reduced pteridine Phenylalanine hydroxylase - Tyrosine hydroxylase - Tryptophan hydroxylase 1.14.17 - reduced ascorbate Dopamine beta hydroxylase 1.14.18-19 - other Tyrosinase - Stearoyl-CoA desaturase-1 1.14.99 - miscellaneous Cyclooxygenase - Heme oxygenase (HMOX1) - Squalene monooxygenase - 17A1 - 21A2

v • d • e Metabolism: amino acid metabolism - synthesis and catabolism enzymes (essential in CAPS) K→acetyl-CoA LYSINE→ Saccharopine dehydrogenase - Glutaryl-CoA dehydrogenase LEUCINE→ Branched chain aminotransferase - Branched-chain alpha-keto acid dehydrogenase complex - Isovaleryl coenzyme A dehydrogenase - Methylcrotonyl-CoA carboxylase - Methylglutaconyl-CoA hydratase - 3-hydroxy-3-methylglutaryl-CoA lyase TRYPTOPHAN→ Indoleamine 2,3-dioxygenase/Tryptophan 2,3-dioxygenase - Arylformamidase - Kynureninase - 3-hydroxyanthranilate oxidase - Aminocarboxymuconate-semialdehyde decarboxylase - Aminomuconate-semialdehyde dehydrogenase PHENYLALANINE→tyrosine→ (see below) G→pyruvate glycine→serine→ Serine hydroxymethyltransferase - Serine dehydratase alanine→ Alanine transaminase cysteine→ D-cysteine desulfhydrase threonine→ L-threonine dehydrogenase G→glutamate HISTIDINE→ Histidine ammonia-lyase - Urocanate hydratase - Formiminotransferase cyclodeaminase proline→ Proline oxidase - Pyrroline-5-carboxylate reductase - 1-Pyrroline-5-carboxylate dehydrogenase/ALDH4A1 - PYCR1 arginine→ Ornithine aminotransferase - Ornithine decarboxylase - Agmatinase →alpha-ketoglutarate→TCA Glutamate dehydrogenase G→propionyl-CoA VALINE→ Branched chain aminotransferase - Branched-chain alpha-keto acid dehydrogenase complex - Enoyl-CoA hydratase - 3-hydroxyisobutyryl-CoA hydrolase - 3-hydroxyisobutyrate dehydrogenase - Methylmalonate semialdehyde dehydrogenase ISOLEUCINE→ Branched chain aminotransferase - Branched-chain alpha-keto acid dehydrogenase complex - 3-hydroxy-2-methylbutyryl-CoA dehydrogenase METHIONINE→ Methionine adenosyltransferase - Adenosylhomocysteinase - Cystathionine beta synthase - Cystathionine gamma-lyase (methionine synthesis: MTR/Homocysteine methyltransferase) THREONINE→ Threonine aldolase →succinyl-CoA→TCA Propionyl-CoA carboxylase - Methylmalonyl-CoA mutase G→fumarate PHENYLALANINE→tyrosine→ Phenylalanine hydroxylase - Tyrosine aminotransferase - 4-Hydroxyphenylpyruvate dioxygenase - Homogentisate 1,2-dioxygenase - Fumarylacetoacetate hydrolase G→oxaloacetate asparagine→aspartate→ Asparaginase/Asparagine synthetase - Aspartate transaminase Other tyrosine→melanin Tyrosinase cysteine+glutamate→glutathione Gamma-glutamylcysteine synthetase - Glutathione synthetase glycine→creatine Guanidinoacetate N-methyltransferase - Creatine kinase glutamate→glutamine Glutamine synthetase - Glutaminase proline+lysine→collagen Prolyl hydroxylase - Lysyl hydroxylase see also disorders, intermediates