5,6,7,8-tetrahydrobiopterin

Three steps

• dopamine quinoneReach from here →
  1. L-Tyrosine,tetrahydrobiopterin:oxygen oxidoreductase (3-hydroxylating)
  2. 3,4-Dihydroxy-L-phenylalanine carboxy-lyase
  3. Peroxidase

  Catalysed by TH, DDC, MPOcytosol, lysosomeOne of 4 routes

• 4-methylsulfanyl-2-oxobutanoateReach from here →
  1. L-phenylalanine,tetrahydrobiopterin:oxygen oxidoreductase(4-hydroxylating)
  2. transport of methionine and tyrosine (cytosol to extracellular)
  3. glyoxylate + L-methionine = 4-methylsulfanyl-2-oxobutanoate + glycine

  Catalysed by PAH, SLC7A5, AADATcytosol, extracellular space

• N-[(S)-lactoyl]-L-phenylalanineReach from here →
  1. L-phenylalanine,tetrahydrobiopterin:oxygen oxidoreductase(4-hydroxylating)
  2. transport of phenylalanine and tyrosine (cytosol to extracellular)
  3. (S)-lactate + L-phenylalanine = N-[(S)-lactoyl]-L-phenylalanine + H2O

  Catalysed by PAH, SLC7A5, CNDP2cytosol, extracellular space

• 2-oxobutanoateReach from here →
  1. L-phenylalanine,tetrahydrobiopterin:oxygen oxidoreductase(4-hydroxylating)
  2. transport of threonine and tyrosine (cytosol to extracellular)
  3. L-threonine ammonia-lyase (2-oxobutanoate-forming)

  Catalysed by PAH, SLC7A5, SDS, SDSLcytosol, extracellular space

• tryptamineReach from here →
  1. L-phenylalanine,tetrahydrobiopterin:oxygen oxidoreductase(4-hydroxylating)
  2. transport of tryptophan and tyrosine (cytosol to extracellular)
  3. L-tryptophan decarboxy-lyase

  Catalysed by PAH, SLC7A5, DDCcytosol, extracellular space

• 2-oxoglutaramateReach from here →
  1. L-phenylalanine,tetrahydrobiopterin:oxygen oxidoreductase(4-hydroxylating)
  2. transport of glutamine and tyrosine (cytosol to extracellular)
  3. L-Glutamine:pyruvate aminotransferase

  Catalysed by PAH, SLC7A5, KYAT1cytosol, extracellular spaceOne of 3 routes

• CHEBI:16856Reach from here →
  1. L-Tryptophan, Tetrahydrobiopterin:Oxygen Oxidoreductase (5-Hydroxylating)
  2. 5-Hydroxy-L-tryptophan decarboxy-lyase
  3. transport of GSH and serotonin (cytosol to extracellular)

  Catalysed by TPH1, TPH2, DDC, SLCO1A2 and 1 morecytosol, extracellular spaceOne of 6 routes

• glyoxylic acidReach from here →
  1. L-phenylalanine,tetrahydrobiopterin:oxygen oxidoreductase(4-hydroxylating)
  2. transport of glycine and tyrosine (cytosol to extracellular)
  3. glycine:oxygen oxidoreductase (deaminating)

  Catalysed by PAH, SLC7A5, DAOcytosol, extracellular space, peroxisome

• oxalic acidReach from here →
  1. L-phenylalanine,tetrahydrobiopterin:oxygen oxidoreductase(4-hydroxylating)
  2. Transport of L-Tyrosine by Atb0 Transporter
  3. transport of chloride and oxalate (cytosol to extracellular)

  Catalysed by PAH, SLC6A14, SLC26A6cytosol, extracellular space

• 2-aminoacrylic acidReach from here →
  1. L-phenylalanine,tetrahydrobiopterin:oxygen oxidoreductase(4-hydroxylating)
  2. thyroid peroxidase (iodine)
  3. thyroid peroxidase (3,5-diiodo-L-tyrosine)

  Catalysed by PAH, TPOcytosol

• (S)-4-hydroxymandelateReach from here →
  1. L-phenylalanine,tetrahydrobiopterin:oxygen oxidoreductase(4-hydroxylating)
  2. L-tyrosine:2-oxoglutarate aminotransferase
  3. 3-(4-hydroxyphenyl)pyruvate + O2 = (S)-4-hydroxymandelate + CO2

  Catalysed by PAH, GOT2, GOT1, TAT and 1 morecytosol

• L-glutamic 5-semialdehydeReach from here →
  1. L-phenylalanine,tetrahydrobiopterin:oxygen oxidoreductase(4-hydroxylating)
  2. transport of ornithine and tyrosine (cytosol to extracellular)
  3. L-Ornithine:2-oxo-acid aminotransferase

  Catalysed by PAH, SLC7A6, OATcytosol, extracellular space, mitochondrial matrix

• dehydroascorbic acidReach from here →
  1. L-Tyrosine,tetrahydrobiopterin:oxygen oxidoreductase (3-hydroxylating)
  2. 3,4-Dihydroxy-L-phenylalanine carboxy-lyase
  3. dopamine,ascorbate:oxygen oxidoreductase (beta-hydroxylating)

  Catalysed by TH, DDC, DBHcytosol

• anilineReach from here →
  1. L-phenylalanine,tetrahydrobiopterin:oxygen oxidoreductase(4-hydroxylating)
  2. Acetylation of Tyrosine
  3. Acetyl-CoA:arylamine N-acetyltransferase

  Catalysed by PAH, NAT2, NAT1cytosol, mitochondrial matrix

• CHEBI:17312Reach from here →
  1. L-phenylalanine,tetrahydrobiopterin:oxygen oxidoreductase(4-hydroxylating)
  2. Acetylation of Tyrosine
  3. N1,N12-diacetylspermine to N1-acetylspermine conversion

  Catalysed by PAHcytosol, mitochondrial matrixOne of 2 routes

• CHEBI:17380Reach from here →
  1. L-phenylalanine,tetrahydrobiopterin:oxygen oxidoreductase(4-hydroxylating)
  2. transport of tryptophan and tyrosine (cytosol to extracellular)
  3. Transport of 3-Hydroxy-L-Kynurenine

  Catalysed by PAH, SLC7A5cytosol, extracellular spaceOne of 2 routes

• (S)-1-pyrroline-5-carboxylateReach from here →
  1. L-phenylalanine,tetrahydrobiopterin:oxygen oxidoreductase(4-hydroxylating)
  2. transport of proline and tyrosine (cytosol to extracellular)
  3. L-proline:(acceptor) oxidoreductase

  Catalysed by PAH, SLC7A5, PRODH2cytosol, extracellular spaceOne of 2 routes

• agmatineReach from here →
  1. L-phenylalanine,tetrahydrobiopterin:oxygen oxidoreductase(4-hydroxylating)
  2. transport of arginine and tyrosine (cytosol to extracellular)
  3. L-arginine carboxy-lyase (agmatine-forming)

  Catalysed by PAH, SLC7A6cytosol, extracellular space, mitochondrial matrix

• AH2Reach from here →
  1. L-phenylalanine,tetrahydrobiopterin:oxygen oxidoreductase(4-hydroxylating)
  2. transport of alanine and tyrosine (cytosol to extracellular)
  3. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O

  Catalysed by PAH, SLC7A5, MOCOScytosol, extracellular spaceOne of 4 routes

• 5-aminolevulinic acidReach from here →
  1. L-phenylalanine,tetrahydrobiopterin:oxygen oxidoreductase(4-hydroxylating)
  2. transport of glycine and tyrosine (cytosol to extracellular)
  3. succinyl-CoA:glycine C-succinyltransferase (decarboxylating)

  Catalysed by PAH, SLC7A5, ALAS1, ALAS2 and 1 morecytosol, extracellular space, mitochondrial matrix

• diiodineReach from here →
  1. L-phenylalanine,tetrahydrobiopterin:oxygen oxidoreductase(4-hydroxylating)
  2. thyroid peroxidase (iodine)
  3. iodide:hydrogen-peroxide oxidoreductase

  Catalysed by PAH, TPOcytosolOne of 2 routes

• indole-3-pyruvateReach from here →
  1. L-phenylalanine,tetrahydrobiopterin:oxygen oxidoreductase(4-hydroxylating)
  2. transport of tryptophan and tyrosine (cytosol to extracellular)
  3. L-tryptophan + O2 + H2O = indole-3-pyruvate + H2O2 + NH4(+)

  Catalysed by PAH, SLC7A5, IL4I1cytosol, extracellular spaceOne of 2 routes

• N-acetylserotoninReach from here →
  1. L-Tryptophan, Tetrahydrobiopterin:Oxygen Oxidoreductase (5-Hydroxylating)
  2. 5-Hydroxy-L-tryptophan decarboxy-lyase
  3. acetyl-CoA:aralkylamine N-acetyltransferase

  Catalysed by TPH1, TPH2, DDC, AANATcytosol

• (7Z,10Z,13Z)-hexadecatrienoyl-CoAReach from here →
  1. L-phenylalanine,tetrahydrobiopterin:oxygen oxidoreductase(4-hydroxylating)
  2. Acetylation of Tyrosine
  3. Fatty Acid Beta Oxidation (C18:3->C16:3), Mitochondrial

  Catalysed by PAH, HADHA, ACADVL, HADHBcytosol, mitochondrial matrix

• Ala-tRNA(Ala)Reach from here →
  1. L-phenylalanine,tetrahydrobiopterin:oxygen oxidoreductase(4-hydroxylating)
  2. transport of alanine and tyrosine (cytosol to extracellular)
  3. L-Alanine:tRNA(Ala) ligase (AMP-forming)

  Catalysed by PAH, SLC7A5, AARS1cytosol, extracellular space

• glycine betaineReach from here →
  1. L-phenylalanine,tetrahydrobiopterin:oxygen oxidoreductase(4-hydroxylating)
  2. Transport of L-Tyrosine by Atb0 Transporter
  3. transport of betaine and chloride (cytosol to extracellular)

  Catalysed by PAH, SLC6A14, SLC6A12cytosol, extracellular spaceOne of 3 routes

• trans-urocanateReach from here →
  1. L-phenylalanine,tetrahydrobiopterin:oxygen oxidoreductase(4-hydroxylating)
  2. transport of histidine and tyrosine (cytosol to extracellular)
  3. L-histidine ammonia-lyase (urocanate-forming)

  Catalysed by PAH, SLC7A6, HALcytosol, extracellular space

• 5-hydroxy-L-tryptophanReach from here →
  1. L-Tryptophan, Tetrahydrobiopterin:Oxygen Oxidoreductase (5-Hydroxylating)
  2. 5-Hydroxy-L-tryptophan decarboxy-lyase
  3. 5-hydroxy-L-tryptophan + H(+) = serotonin + CO2

  Catalysed by TPH1, TPH2, DDCcytosolOne of 2 routes

• 4-methyl-2-oxopentanoateReach from here →
  1. L-Tyrosine,tetrahydrobiopterin:oxygen oxidoreductase (3-hydroxylating)
  2. solute carrier (L-dopa)
  3. L-Leucine:2-oxoglutarate aminotransferase

  Catalysed by TH, SLC3A2, SLC7A5, BCAT2cytosol, extracellular space, mitochondrial matrixOne of 4 routes

• CHEBI:17927Reach from here →
  1. L-phenylalanine,tetrahydrobiopterin:oxygen oxidoreductase(4-hydroxylating)
  2. transport of arginine and tyrosine (cytosol to extracellular)
  3. Transport by Y+Lat-2 into Extracellular Space

  Catalysed by PAH, SLC7A6, SLC3A2cytosol, extracellular spaceOne of 2 routes

• 3-phenylpyruvateReach from here →
  1. L-phenylalanine,tetrahydrobiopterin:oxygen oxidoreductase(4-hydroxylating)
  2. transport of phenylalanine and tyrosine (cytosol to extracellular)
  3. Phenylalanine Transaminase, Mitochondrial

  Catalysed by PAH, SLC7A5, GOT2, TATcytosol, extracellular space, mitochondrial matrixOne of 5 routes

• CHEBI:18095Reach from here →
  1. L-phenylalanine,tetrahydrobiopterin:oxygen oxidoreductase(4-hydroxylating)
  2. transport of alanine and tyrosine (cytosol to extracellular)
  3. Transport of Hydroxy-Proline

  Catalysed by PAH, SLC7A5, SLC1A4cytosol, extracellular spaceOne of 5 routes

• N-carbamoyl-beta-alanineReach from here →
  1. L-phenylalanine,tetrahydrobiopterin:oxygen oxidoreductase(4-hydroxylating)
  2. Transport of L-Tyrosine by Atb0 Transporter
  3. Transport of 3-Ureidopropionate

  Catalysed by PAH, SLC6A14cytosol, extracellular spaceOne of 2 routes

• N(1)-(5-phospho-D-ribosyl)glycinamideReach from here →
  1. L-phenylalanine,tetrahydrobiopterin:oxygen oxidoreductase(4-hydroxylating)
  2. transport of glycine and tyrosine (cytosol to extracellular)
  3. 5-Phospho-D-ribosylamine:glycine ligase (ADP-forming)

  Catalysed by PAH, SLC7A5, GARTcytosol, extracellular space

• 2-phenylethylamineReach from here →
  1. L-phenylalanine,tetrahydrobiopterin:oxygen oxidoreductase(4-hydroxylating)
  2. transport of phenylalanine and tyrosine (cytosol to extracellular)
  3. L-phenylalanine carboxy-lyase (phenylethylamine-forming)

  Catalysed by PAH, SLC7A5, DDCcytosol, extracellular space

• 2-formamido-N(1)-(5-phospho-D-ribosyl)acetamidineReach from here →
  1. L-phenylalanine,tetrahydrobiopterin:oxygen oxidoreductase(4-hydroxylating)
  2. transport of glutamine and tyrosine (cytosol to extracellular)
  3. 5'-Phosphoribosylformylglycinamide:L-glutamine amido-ligase (ADP-forming)

  Catalysed by PAH, SLC7A5, PFAScytosol, extracellular space

• ophthalmateReach from here →
  1. L-phenylalanine,tetrahydrobiopterin:oxygen oxidoreductase(4-hydroxylating)
  2. transport of glycine and tyrosine (cytosol to extracellular)
  3. gamma-L-glutamyl-(2S)-2-aminobutanoate + glycine + ATP = ophthalmate + ADP + phosphate + H(+)

  Catalysed by PAH, SLC7A5, GSScytosol, extracellular space

• S-methylmercury-L-cysteineReach from here →
  1. L-Tyrosine,tetrahydrobiopterin:oxygen oxidoreductase (3-hydroxylating)
  2. solute carrier (L-dopa)
  3. S-methylmercury-L-cysteine(in) + L-leucine(out) = S-methylmercury-L-cysteine(out) + L-leucine(in)

  Catalysed by TH, SLC3A2, SLC7A5, SLC7A8cytosol, extracellular space, inner side of the membrane, outer side of the membraneOne of 6 routes

• 2-aminoisobutanoateReach from here →
  1. L-phenylalanine,tetrahydrobiopterin:oxygen oxidoreductase(4-hydroxylating)
  2. transport of alanine and tyrosine (cytosol to extracellular)
  3. 2-aminoisobutanoate(out) + L-alanine(in) = 2-aminoisobutanoate(in) + L-alanine(out)

  Catalysed by PAH, SLC7A5, SLC7A10cytosol, extracellular space, inner side of the membrane, outer side of the membraneOne of 2 routes

• N(omega),N('omega)-dimethyl-L-arginineReach from here →
  1. L-phenylalanine,tetrahydrobiopterin:oxygen oxidoreductase(4-hydroxylating)
  2. transport of glycine and tyrosine (cytosol to extracellular)
  3. N(omega),N('omega)-dimethyl-L-arginine + glyoxylate = 5-(3,3'-dimethylguanidino)-2-oxopentanoate + glycine

  Catalysed by PAH, SLC7A5, AGXT2cytosol, extracellular spaceOne of 2 routes

• 5,6,7,8-tetrahydrofolic acidReach from here →
  1. L-phenylalanine,tetrahydrobiopterin:oxygen oxidoreductase(4-hydroxylating)
  2. transport of glycine and tyrosine (cytosol to extracellular)
  3. 5,10-Methylenetetrahydrofolate:glycine hydroxymethyltransferase

  Catalysed by PAH, SLC7A5, SHMT1cytosol, extracellular spaceOne of 2 routes

• S-disulfanyl-L-cysteineReach from here →
  1. L-phenylalanine,tetrahydrobiopterin:oxygen oxidoreductase(4-hydroxylating)
  2. transport of cysteine and tyrosine (cytosol to extracellular)
  3. S-sulfanyl-L-cysteine + L-cysteine = S-disulfanyl-L-cysteine + L-alanine

  Catalysed by PAH, SLC7A5, CARS2cytosol, extracellular space

• (5Z)-dodecenoyl-CoAReach from here →
  1. L-phenylalanine,tetrahydrobiopterin:oxygen oxidoreductase(4-hydroxylating)
  2. Acetylation of Tyrosine
  3. acetyl-CoA C-acyltransferase (3-oxomyrist-7-enoyl-CoA)

  Catalysed by PAH, HADHA, ACAA2, HADHBcytosol, mitochondrial matrixOne of 2 routes

• (11Z)-docosenoyl-CoA(4-)Reach from here →
  1. L-phenylalanine,tetrahydrobiopterin:oxygen oxidoreductase(4-hydroxylating)
  2. Acetylation of Tyrosine
  3. long-chain-fatty-acid-CoA ligase (cis-cetoleic acid)

  Catalysed by PAH, SLC27A2, ACSL4, ACSL3 and 5 morecytosol, mitochondrial matrixOne of 5 routes

• (7Z)-tetradecenoyl-CoAReach from here →
  1. L-phenylalanine,tetrahydrobiopterin:oxygen oxidoreductase(4-hydroxylating)
  2. Acetylation of Tyrosine
  3. long-chain-fatty-acid-CoA ligase ((7Z)-tetradecenoic acid)

  Catalysed by PAH, SLC27A2, ACSL4, ACSL3 and 5 morecytosol, mitochondrial matrixOne of 8 routes

• serotonin O-sulfateReach from here →
  1. L-Tryptophan, Tetrahydrobiopterin:Oxygen Oxidoreductase (5-Hydroxylating)
  2. 5-Hydroxy-L-tryptophan decarboxy-lyase
  3. serotonin + 3'-phosphoadenylyl sulfate = serotonin O-sulfate + adenosine 3',5'-bisphosphate + H(+)

  Catalysed by TPH1, TPH2, DDC, SULT1A3 and 1 morecytosol

• (4Z,7Z,10Z,13Z)-hexadecatetraenoyl-CoAReach from here →
  1. L-phenylalanine,tetrahydrobiopterin:oxygen oxidoreductase(4-hydroxylating)
  2. Acetylation of Tyrosine
  3. Fatty Acid Beta Oxidation (C18:4->C16:4), Mitochondrial

  Catalysed by PAH, HADHA, ACADVL, HADHBcytosol, mitochondrial matrixOne of 3 routes

• (7Z,10Z)-hexadecadienoyl-CoAReach from here →
  1. L-phenylalanine,tetrahydrobiopterin:oxygen oxidoreductase(4-hydroxylating)
  2. Acetylation of Tyrosine
  3. Fatty Acid Beta Oxidation Trans (C18:2->C16:2), Mitochondrial

  Catalysed by PAH, HADHA, ACADVL, HADHBcytosol, mitochondrial matrix

• (4Z,7Z)-decadienoyl-CoAReach from here →
  1. L-phenylalanine,tetrahydrobiopterin:oxygen oxidoreductase(4-hydroxylating)
  2. Acetylation of Tyrosine
  3. Fatty Acid Beta Oxidation (C12:3->C10:2), Mitochondrial

  Catalysed by PAH, ECHS1, ACAA2, HADHcytosol, mitochondrial matrixOne of 2 routes

• (5Z)-octenoyl-CoAReach from here →
  1. L-phenylalanine,tetrahydrobiopterin:oxygen oxidoreductase(4-hydroxylating)
  2. Acetylation of Tyrosine
  3. Fatty Acid Beta Oxidation (C10:2->C8:1), Mitochondrial

  Catalysed by PAH, ECHS1, ACAA2, HADHcytosol, mitochondrial matrixOne of 2 routes