(6R)-L-erythro-5,6,7,8-tetrahydrobiopterin

Three steps

• N-dodecanoylglycineReach from here →
  1. (6R)-L-erythro-5,6,7,8-tetrahydrobiopterin + L-phenylalanine + O2 = (4aS,6R)-4a-hydroxy-L-erythro-5,6,7,8-tetrahydrobiopterin + L-tyrosine
  2. L-tyrosine + glyoxylate = 3-(4-hydroxyphenyl)pyruvate + glycine
  3. dodecanoyl-CoA + glycine = N-dodecanoylglycine + CoA + H(+)

  Catalysed by PAH, AADAT, GLYATL2

• N-decanoylglycineReach from here →
  1. (6R)-L-erythro-5,6,7,8-tetrahydrobiopterin + L-phenylalanine + O2 = (4aS,6R)-4a-hydroxy-L-erythro-5,6,7,8-tetrahydrobiopterin + L-tyrosine
  2. L-tyrosine + glyoxylate = 3-(4-hydroxyphenyl)pyruvate + glycine
  3. decanoyl-CoA + glycine = N-decanoylglycine + CoA + H(+)

  Catalysed by PAH, AADAT, GLYATL2

• N-octanoylglycineReach from here →
  1. (6R)-L-erythro-5,6,7,8-tetrahydrobiopterin + L-phenylalanine + O2 = (4aS,6R)-4a-hydroxy-L-erythro-5,6,7,8-tetrahydrobiopterin + L-tyrosine
  2. L-tyrosine + glyoxylate = 3-(4-hydroxyphenyl)pyruvate + glycine
  3. octanoyl-CoA + glycine = N-octanoylglycine + CoA + H(+)

  Catalysed by PAH, AADAT, GLYATL2

• N(1)-(5-phospho-beta-D-ribosyl)glycinamideReach from here →
  1. (6R)-L-erythro-5,6,7,8-tetrahydrobiopterin + L-phenylalanine + O2 = (4aS,6R)-4a-hydroxy-L-erythro-5,6,7,8-tetrahydrobiopterin + L-tyrosine
  2. L-tyrosine + glyoxylate = 3-(4-hydroxyphenyl)pyruvate + glycine
  3. 5-phospho-beta-D-ribosylamine + glycine + ATP = N(1)-(5-phospho-beta-D-ribosyl)glycinamide + ADP + phosphate + H(+)

  Catalysed by PAH, AADAT, GART

• L-cystine-bis-glycineReach from here →
  1. (6R)-L-erythro-5,6,7,8-tetrahydrobiopterin + L-phenylalanine + O2 = (4aS,6R)-4a-hydroxy-L-erythro-5,6,7,8-tetrahydrobiopterin + L-tyrosine
  2. L-tyrosine + glyoxylate = 3-(4-hydroxyphenyl)pyruvate + glycine
  3. L-cystine-bis-glycine + 2 H2O = L-cystine + 2 glycine

  Catalysed by PAH, AADAT, DPEP1, DPEP2

• glycyldehydrophenylalanineReach from here →
  1. (6R)-L-erythro-5,6,7,8-tetrahydrobiopterin + L-phenylalanine + O2 = (4aS,6R)-4a-hydroxy-L-erythro-5,6,7,8-tetrahydrobiopterin + L-tyrosine
  2. L-tyrosine + glyoxylate = 3-(4-hydroxyphenyl)pyruvate + glycine
  3. glycyldehydrophenylalanine + H2O = 2,3-didehydrophenylalanine + glycine

  Catalysed by PAH, AADAT, DPEP1

• kinetensinReach from here →
  1. (6R)-L-erythro-5,6,7,8-tetrahydrobiopterin + L-tryptophan + O2 = 5-hydroxy-L-tryptophan + (4aS,6R)-4a-hydroxy-L-erythro-5,6,7,8-tetrahydrobiopterin
  2. (6R)-L-erythro-5,6,7,8-tetrahydrobiopterin + L-phenylalanine + O2 = (4aS,6R)-4a-hydroxy-L-erythro-5,6,7,8-tetrahydrobiopterin + L-tyrosine
  3. Chymase

  Catalysed by TPH1, TPH2, PAH, CMA1extracellular space

• an N-(fatty acyl)-glycineReach from here →
  1. (6R)-L-erythro-5,6,7,8-tetrahydrobiopterin + L-phenylalanine + O2 = (4aS,6R)-4a-hydroxy-L-erythro-5,6,7,8-tetrahydrobiopterin + L-tyrosine
  2. L-tyrosine + glyoxylate = 3-(4-hydroxyphenyl)pyruvate + glycine
  3. a fatty acyl-CoA + glycine = an N-(fatty acyl)-glycine + CoA + H(+)

  Catalysed by PAH, AADAT, GLYATL2

• N-(9Z,12Z-octadecadienoyl)-glycineReach from here →
  1. (6R)-L-erythro-5,6,7,8-tetrahydrobiopterin + L-phenylalanine + O2 = (4aS,6R)-4a-hydroxy-L-erythro-5,6,7,8-tetrahydrobiopterin + L-tyrosine
  2. L-tyrosine + glyoxylate = 3-(4-hydroxyphenyl)pyruvate + glycine
  3. (9Z,12Z)-octadecadienoyl-CoA + glycine = N-(9Z,12Z-octadecadienoyl)-glycine + CoA + H(+)

  Catalysed by PAH, AADAT, GLYATL2

• 4a-hydroxytetrahydrobiopterinReach from here →
  1. (6R)-L-erythro-5,6,7,8-tetrahydrobiopterin + L-tryptophan + O2 = 5-hydroxy-L-tryptophan + (4aS,6R)-4a-hydroxy-L-erythro-5,6,7,8-tetrahydrobiopterin
  2. (6R)-L-erythro-5,6,7,8-tetrahydrobiopterin + L-tryptophan + O2 = 5-hydroxy-L-tryptophan + (4aS,6R)-4a-hydroxy-L-erythro-5,6,7,8-tetrahydrobiopterin
  3. L-tryptophan,tetrahydrobiopterin:oxygen oxidoreductase(5-hydroxylating)

  Catalysed by TPH1, TPH2cytosolOne of 2 routes

• CHEBI:15570Reach from here →
  1. (6R)-L-erythro-5,6,7,8-tetrahydrobiopterin + L-phenylalanine + O2 = (4aS,6R)-4a-hydroxy-L-erythro-5,6,7,8-tetrahydrobiopterin + L-tyrosine
  2. L-tyrosine + glyoxylate = 3-(4-hydroxyphenyl)pyruvate + glycine
  3. transport of D-alanine and glycine (cytosol to extracellular)

  Catalysed by PAH, AADAT, SLC3A2, SLC7A10extracellular space, cytosolOne of 2 routes

• (S)-malateReach from here →
  1. (6R)-L-erythro-5,6,7,8-tetrahydrobiopterin(out) + a dicarboxylate(in) = (6R)-L-erythro-5,6,7,8-tetrahydrobiopterin(in) + a dicarboxylate(out)
  2. a dicarboxylate(in) + sulfate(out) = a dicarboxylate(out) + sulfate(in)
  3. transport of malate and sulfate (cytosol to mitochondria)

  Catalysed by SLC22A6, SLC22A8, SLC25A35, SLC25A34 and 1 moreinner side of the membrane, outer side of the membrane, mitochondrial matrix, cytosolOne of 4 routes

• L-leucineReach from here →
  1. (6R)-L-erythro-5,6,7,8-tetrahydrobiopterin + L-tryptophan + O2 = 5-hydroxy-L-tryptophan + (4aS,6R)-4a-hydroxy-L-erythro-5,6,7,8-tetrahydrobiopterin
  2. (6R)-L-erythro-5,6,7,8-tetrahydrobiopterin + L-tryptophan + O2 = 5-hydroxy-L-tryptophan + (4aS,6R)-4a-hydroxy-L-erythro-5,6,7,8-tetrahydrobiopterin
  3. transport of leucine and tryptophan (cytosol to extracellular)

  Catalysed by TPH1, TPH2, SLC7A5extracellular space, cytosolOne of 17 routes

• CHEBI:15611Reach from here →
  1. (6R)-L-erythro-5,6,7,8-tetrahydrobiopterin + L-phenylalanine + O2 = (4aS,6R)-4a-hydroxy-L-erythro-5,6,7,8-tetrahydrobiopterin + L-tyrosine
  2. L-tyrosine + glyoxylate = 3-(4-hydroxyphenyl)pyruvate + glycine
  3. S-Adenosyl-L-methionine:glycine N-methyltransferase

  Catalysed by PAH, AADAT, GNMTcytosol

• CHEBI:15699Reach from here →
  1. (6R)-L-erythro-5,6,7,8-tetrahydrobiopterin + L-tryptophan + O2 = 5-hydroxy-L-tryptophan + (4aS,6R)-4a-hydroxy-L-erythro-5,6,7,8-tetrahydrobiopterin
  2. (6R)-L-erythro-5,6,7,8-tetrahydrobiopterin + L-tryptophan + O2 = 5-hydroxy-L-tryptophan + (4aS,6R)-4a-hydroxy-L-erythro-5,6,7,8-tetrahydrobiopterin
  3. transport of homoserine and tryptophan (cytosol to extracellular)

  Catalysed by TPH1, TPH2, SLC7A5extracellular space, cytosolOne of 10 routes

• CHEBI:15765Reach from here →
  1. (6R)-L-erythro-5,6,7,8-tetrahydrobiopterin + L-tyrosine + O2 = (4aS,6R)-4a-hydroxy-L-erythro-5,6,7,8-tetrahydrobiopterin + L-dopa
  2. 2 L-dopa + O2 = 2 L-dopaquinone + 2 H2O
  3. 2-carboxy-2,3-dihydro-5,6-dihydroxyindole to L-dopa conversion

  Catalysed by TH, TYRcytosolOne of 2 routes

• malonateReach from here →
  1. (6R)-L-erythro-5,6,7,8-tetrahydrobiopterin(out) + a dicarboxylate(in) = (6R)-L-erythro-5,6,7,8-tetrahydrobiopterin(in) + a dicarboxylate(out)
  2. a dicarboxylate(in) + sulfate(out) = a dicarboxylate(out) + sulfate(in)
  3. malonate(in) + sulfate(out) = malonate(out) + sulfate(in)

  Catalysed by SLC22A6, SLC22A8, SLC25A35, SLC25A34 and 3 moreinner side of the membrane, outer side of the membraneOne of 2 routes

• N-carbamoyl-L-aspartic acidReach from here →
  1. (6R)-L-erythro-5,6,7,8-tetrahydrobiopterin + L-phenylalanine + O2 = (4aS,6R)-4a-hydroxy-L-erythro-5,6,7,8-tetrahydrobiopterin + L-tyrosine
  2. L-tyrosine(out) + L-aspartate(in) = L-tyrosine(in) + L-aspartate(out)
  3. carbamoyl-phosphate:L-aspartate carbamoyltransferase

  Catalysed by PAH, SLC7A13, CADinner side of the membrane, outer side of the membrane, cytosol

• CHEBI:15971Reach from here →
  1. (6R)-L-erythro-5,6,7,8-tetrahydrobiopterin + L-tryptophan + O2 = 5-hydroxy-L-tryptophan + (4aS,6R)-4a-hydroxy-L-erythro-5,6,7,8-tetrahydrobiopterin
  2. (6R)-L-erythro-5,6,7,8-tetrahydrobiopterin + L-tryptophan + O2 = 5-hydroxy-L-tryptophan + (4aS,6R)-4a-hydroxy-L-erythro-5,6,7,8-tetrahydrobiopterin
  3. transport of histidine and tryptophan (cytosol to extracellular)

  Catalysed by TPH1, TPH2, SLC7A6extracellular space, cytosolOne of 6 routes

• homogentisateReach from here →
  1. (6R)-L-erythro-5,6,7,8-tetrahydrobiopterin + L-phenylalanine + O2 = (4aS,6R)-4a-hydroxy-L-erythro-5,6,7,8-tetrahydrobiopterin + L-tyrosine
  2. L-tyrosine + 2-oxoglutarate = 3-(4-hydroxyphenyl)pyruvate + L-glutamate
  3. 4-Hydroxyphenylpyruvate:oxygen oxidoreductase (hydroxylating,decarboxylating)

  Catalysed by PAH, TAT, HPDcytosolOne of 2 routes

• ureaReach from here →
  1. (6R)-L-erythro-5,6,7,8-tetrahydrobiopterin(out) + a dicarboxylate(in) = (6R)-L-erythro-5,6,7,8-tetrahydrobiopterin(in) + a dicarboxylate(out)
  2. urate(out) + a dicarboxylate(in) = urate(in) + a dicarboxylate(out)
  3. Peroxidase

  Catalysed by SLC22A6, SLC22A8, MPOinner side of the membrane, outer side of the membrane, nucleusOne of 3 routes

• L-cystineReach from here →
  1. (6R)-L-erythro-5,6,7,8-tetrahydrobiopterin + L-phenylalanine + O2 = (4aS,6R)-4a-hydroxy-L-erythro-5,6,7,8-tetrahydrobiopterin + L-tyrosine
  2. L-tyrosine(out) + L-aspartate(in) = L-tyrosine(in) + L-aspartate(out)
  3. L-cystine(out) + L-aspartate(in) = L-cystine(in) + L-aspartate(out)

  Catalysed by PAH, SLC7A13inner side of the membrane, outer side of the membraneOne of 2 routes

• L-citrullineReach from here →
  1. (6R)-L-erythro-5,6,7,8-tetrahydrobiopterin + L-tryptophan + O2 = 5-hydroxy-L-tryptophan + (4aS,6R)-4a-hydroxy-L-erythro-5,6,7,8-tetrahydrobiopterin
  2. (6R)-L-erythro-5,6,7,8-tetrahydrobiopterin + L-tryptophan + O2 = 5-hydroxy-L-tryptophan + (4aS,6R)-4a-hydroxy-L-erythro-5,6,7,8-tetrahydrobiopterin
  3. transport of citrulline and tryptophan (cytosol to extracellular)

  Catalysed by TPH1, TPH2, SLC7A9, SLC3A1 and 1 moreextracellular space, cytosolOne of 4 routes

• L-valineReach from here →
  1. (6R)-L-erythro-5,6,7,8-tetrahydrobiopterin + L-tryptophan + O2 = 5-hydroxy-L-tryptophan + (4aS,6R)-4a-hydroxy-L-erythro-5,6,7,8-tetrahydrobiopterin
  2. (6R)-L-erythro-5,6,7,8-tetrahydrobiopterin + L-tryptophan + O2 = 5-hydroxy-L-tryptophan + (4aS,6R)-4a-hydroxy-L-erythro-5,6,7,8-tetrahydrobiopterin
  3. transport of tryptophan and valine (cytosol to extracellular)

  Catalysed by TPH1, TPH2, SLC7A5extracellular space, cytosolOne of 15 routes

• oxaloacetateReach from here →
  1. (6R)-L-erythro-5,6,7,8-tetrahydrobiopterin + L-phenylalanine + O2 = (4aS,6R)-4a-hydroxy-L-erythro-5,6,7,8-tetrahydrobiopterin + L-tyrosine
  2. L-tyrosine(out) + L-aspartate(in) = L-tyrosine(in) + L-aspartate(out)
  3. L-Aspartate:2-oxoglutarate aminotransferase

  Catalysed by PAH, SLC7A13, GOT2inner side of the membrane, outer side of the membrane, mitochondrial matrixOne of 5 routes

• L-arginineReach from here →
  1. (6R)-L-erythro-5,6,7,8-tetrahydrobiopterin + L-tryptophan + O2 = 5-hydroxy-L-tryptophan + (4aS,6R)-4a-hydroxy-L-erythro-5,6,7,8-tetrahydrobiopterin
  2. (6R)-L-erythro-5,6,7,8-tetrahydrobiopterin + L-tryptophan + O2 = 5-hydroxy-L-tryptophan + (4aS,6R)-4a-hydroxy-L-erythro-5,6,7,8-tetrahydrobiopterin
  3. transport of arginine and tryptophan (cytosol to extracellular)

  Catalysed by TPH1, TPH2, SLC7A6extracellular space, cytosolOne of 8 routes

• CHEBI:16523Reach from here →
  1. (6R)-L-erythro-5,6,7,8-tetrahydrobiopterin + L-phenylalanine + O2 = (4aS,6R)-4a-hydroxy-L-erythro-5,6,7,8-tetrahydrobiopterin + L-tyrosine
  2. L-tyrosine + glyoxylate = 3-(4-hydroxyphenyl)pyruvate + glycine
  3. transport of D-serine and glycine (cytosol to extracellular)

  Catalysed by PAH, AADAT, SLC3A2, SLC7A10extracellular space, cytosolOne of 2 routes

• protein polypeptide chainReach from here →
  1. (6R)-L-erythro-5,6,7,8-tetrahydrobiopterin + L-tryptophan + O2 = 5-hydroxy-L-tryptophan + (4aS,6R)-4a-hydroxy-L-erythro-5,6,7,8-tetrahydrobiopterin
  2. (6R)-L-erythro-5,6,7,8-tetrahydrobiopterin + L-tryptophan + O2 = 5-hydroxy-L-tryptophan + (4aS,6R)-4a-hydroxy-L-erythro-5,6,7,8-tetrahydrobiopterin
  3. PROTEIN_BS

  Catalysed by TPH1, TPH2cytosolOne of 4 routes

• 2-phenylacetamideReach from here →
  1. (6R)-L-erythro-5,6,7,8-tetrahydrobiopterin + L-tryptophan + O2 = 5-hydroxy-L-tryptophan + (4aS,6R)-4a-hydroxy-L-erythro-5,6,7,8-tetrahydrobiopterin
  2. (6R)-L-erythro-5,6,7,8-tetrahydrobiopterin + L-phenylalanine + O2 = (4aS,6R)-4a-hydroxy-L-erythro-5,6,7,8-tetrahydrobiopterin + L-tyrosine
  3. L-phenylalanine:oxygen 2-oxidoreductase (decarboxylating)

  Catalysed by TPH1, TPH2, PAH, EPXcytosol

• (S)-lactateReach from here →
  1. (6R)-L-erythro-5,6,7,8-tetrahydrobiopterin(out) + a dicarboxylate(in) = (6R)-L-erythro-5,6,7,8-tetrahydrobiopterin(in) + a dicarboxylate(out)
  2. prostaglandin F2alpha(out) + a dicarboxylate(in) = prostaglandin F2alpha(in) + a dicarboxylate(out)
  3. prostaglandin F2alpha(out) + 2 (S)-lactate(in) = prostaglandin F2alpha(in) + 2 (S)-lactate(out)

  Catalysed by SLC22A6, SLC22A8, SLCO2A1inner side of the membrane, outer side of the membraneOne of 5 routes

• quinolinic acidReach from here →
  1. (6R)-L-erythro-5,6,7,8-tetrahydrobiopterin(out) + a dicarboxylate(in) = (6R)-L-erythro-5,6,7,8-tetrahydrobiopterin(in) + a dicarboxylate(out)
  2. a dicarboxylate(in) + sulfate(out) = a dicarboxylate(out) + sulfate(in)
  3. Transport of Quinolinic Acid

  Catalysed by SLC22A6, SLC22A8, SLC25A35, SLC25A34inner side of the membrane, outer side of the membrane, extracellular space, cytosolOne of 2 routes

• dopamine quinoneReach from here →
  1. (6R)-L-erythro-5,6,7,8-tetrahydrobiopterin + L-tyrosine + O2 = (4aS,6R)-4a-hydroxy-L-erythro-5,6,7,8-tetrahydrobiopterin + L-dopa
  2. L-dopa + H(+) = dopamine + CO2
  3. Peroxidase

  Catalysed by TH, DDC, MPOlysosomeOne of 4 routes

• N-[(S)-lactoyl]-L-phenylalanineReach from here →
  1. (6R)-L-erythro-5,6,7,8-tetrahydrobiopterin + L-tryptophan + O2 = 5-hydroxy-L-tryptophan + (4aS,6R)-4a-hydroxy-L-erythro-5,6,7,8-tetrahydrobiopterin
  2. (6R)-L-erythro-5,6,7,8-tetrahydrobiopterin + L-phenylalanine + O2 = (4aS,6R)-4a-hydroxy-L-erythro-5,6,7,8-tetrahydrobiopterin + L-tyrosine
  3. (S)-lactate + L-phenylalanine = N-[(S)-lactoyl]-L-phenylalanine + H2O

  Catalysed by TPH1, TPH2, PAH, CNDP2

• tryptamineReach from here →
  1. (6R)-L-erythro-5,6,7,8-tetrahydrobiopterin + L-tryptophan + O2 = 5-hydroxy-L-tryptophan + (4aS,6R)-4a-hydroxy-L-erythro-5,6,7,8-tetrahydrobiopterin
  2. (6R)-L-erythro-5,6,7,8-tetrahydrobiopterin + L-tryptophan + O2 = 5-hydroxy-L-tryptophan + (4aS,6R)-4a-hydroxy-L-erythro-5,6,7,8-tetrahydrobiopterin
  3. L-tryptophan decarboxy-lyase

  Catalysed by TPH1, TPH2, DDCcytosol

• CHEBI:16856Reach from here →
  1. (6R)-L-erythro-5,6,7,8-tetrahydrobiopterin + L-tryptophan + O2 = 5-hydroxy-L-tryptophan + (4aS,6R)-4a-hydroxy-L-erythro-5,6,7,8-tetrahydrobiopterin
  2. 5-hydroxy-L-tryptophan + H(+) = serotonin + CO2
  3. transport of GSH and serotonin (cytosol to extracellular)

  Catalysed by TPH1, TPH2, DDC, SLCO1A2 and 1 moreextracellular space, cytosolOne of 9 routes

• CHEBI:16857Reach from here →
  1. (6R)-L-erythro-5,6,7,8-tetrahydrobiopterin + L-tryptophan + O2 = 5-hydroxy-L-tryptophan + (4aS,6R)-4a-hydroxy-L-erythro-5,6,7,8-tetrahydrobiopterin
  2. (6R)-L-erythro-5,6,7,8-tetrahydrobiopterin + L-tryptophan + O2 = 5-hydroxy-L-tryptophan + (4aS,6R)-4a-hydroxy-L-erythro-5,6,7,8-tetrahydrobiopterin
  3. transport of threonine and tryptophan (cytosol to extracellular)

  Catalysed by TPH1, TPH2, SLC7A5extracellular space, cytosolOne of 12 routes

• glyoxylic acidReach from here →
  1. (6R)-L-erythro-5,6,7,8-tetrahydrobiopterin + L-phenylalanine + O2 = (4aS,6R)-4a-hydroxy-L-erythro-5,6,7,8-tetrahydrobiopterin + L-tyrosine
  2. L-tyrosine + glyoxylate = 3-(4-hydroxyphenyl)pyruvate + glycine
  3. glycine:oxygen oxidoreductase (deaminating)

  Catalysed by PAH, AADAT, DAOperoxisome

• N-acetyl-L-aspartateReach from here →
  1. (6R)-L-erythro-5,6,7,8-tetrahydrobiopterin + L-phenylalanine + O2 = (4aS,6R)-4a-hydroxy-L-erythro-5,6,7,8-tetrahydrobiopterin + L-tyrosine
  2. L-tyrosine(out) + L-aspartate(in) = L-tyrosine(in) + L-aspartate(out)
  3. acetyl-CoA:L-aspartate N-acetyltransferase

  Catalysed by PAH, SLC7A13, NAT8Linner side of the membrane, outer side of the membrane, mitochondrial matrixOne of 2 routes

• oxalic acidReach from here →
  1. (6R)-L-erythro-5,6,7,8-tetrahydrobiopterin(out) + a dicarboxylate(in) = (6R)-L-erythro-5,6,7,8-tetrahydrobiopterin(in) + a dicarboxylate(out)
  2. a dicarboxylate(in) + sulfate(out) = a dicarboxylate(out) + sulfate(in)
  3. transport of oxalate and sulfate (cytosol to extracellular)

  Catalysed by SLC22A6, SLC22A8, SLC25A35, SLC25A34 and 1 moreinner side of the membrane, outer side of the membrane, extracellular space, cytosol

• L-isoleucineReach from here →
  1. (6R)-L-erythro-5,6,7,8-tetrahydrobiopterin + L-tryptophan + O2 = 5-hydroxy-L-tryptophan + (4aS,6R)-4a-hydroxy-L-erythro-5,6,7,8-tetrahydrobiopterin
  2. (6R)-L-erythro-5,6,7,8-tetrahydrobiopterin + L-tryptophan + O2 = 5-hydroxy-L-tryptophan + (4aS,6R)-4a-hydroxy-L-erythro-5,6,7,8-tetrahydrobiopterin
  3. transport of isoleucine and tryptophan (cytosol to extracellular)

  Catalysed by TPH1, TPH2, SLC7A5extracellular space, cytosolOne of 13 routes

• CHEBI:17203Reach from here →
  1. (6R)-L-erythro-5,6,7,8-tetrahydrobiopterin + L-tryptophan + O2 = 5-hydroxy-L-tryptophan + (4aS,6R)-4a-hydroxy-L-erythro-5,6,7,8-tetrahydrobiopterin
  2. (6R)-L-erythro-5,6,7,8-tetrahydrobiopterin + L-tryptophan + O2 = 5-hydroxy-L-tryptophan + (4aS,6R)-4a-hydroxy-L-erythro-5,6,7,8-tetrahydrobiopterin
  3. transport of proline and tryptophan (cytosol to extracellular)

  Catalysed by TPH1, TPH2, ABCA1, SLC7A5extracellular space, cytosolOne of 10 routes

• CHEBI:17208Reach from here →
  1. (6R)-L-erythro-5,6,7,8-tetrahydrobiopterin(out) + a dicarboxylate(in) = (6R)-L-erythro-5,6,7,8-tetrahydrobiopterin(in) + a dicarboxylate(out)
  2. urate(out) + a dicarboxylate(in) = urate(in) + a dicarboxylate(out)
  3. ascorbate to monodehydroascorbate conversion

  Catalysed by SLC22A6, SLC22A8inner side of the membrane, outer side of the membrane, cytosol

• (S)-4-hydroxymandelateReach from here →
  1. (6R)-L-erythro-5,6,7,8-tetrahydrobiopterin + L-phenylalanine + O2 = (4aS,6R)-4a-hydroxy-L-erythro-5,6,7,8-tetrahydrobiopterin + L-tyrosine
  2. L-tyrosine + 2-oxoglutarate = 3-(4-hydroxyphenyl)pyruvate + L-glutamate
  3. 3-(4-hydroxyphenyl)pyruvate + O2 = (S)-4-hydroxymandelate + CO2

  Catalysed by PAH, TAT, HPDL

• dehydroascorbic acidReach from here →
  1. (6R)-L-erythro-5,6,7,8-tetrahydrobiopterin + L-tyrosine + O2 = (4aS,6R)-4a-hydroxy-L-erythro-5,6,7,8-tetrahydrobiopterin + L-dopa
  2. L-dopa + H(+) = dopamine + CO2
  3. dopamine,ascorbate:oxygen oxidoreductase (beta-hydroxylating)

  Catalysed by TH, DDC, DBHcytosol

• sulfiteReach from here →
  1. (6R)-L-erythro-5,6,7,8-tetrahydrobiopterin(out) + a dicarboxylate(in) = (6R)-L-erythro-5,6,7,8-tetrahydrobiopterin(in) + a dicarboxylate(out)
  2. a dicarboxylate(in) + sulfate(out) = a dicarboxylate(out) + sulfate(in)
  3. sulfite(in) + sulfate(out) = sulfite(out) + sulfate(in)

  Catalysed by SLC22A6, SLC22A8, SLC25A35, SLC25A34 and 2 moreinner side of the membrane, outer side of the membraneOne of 2 routes

• CHEBI:17380Reach from here →
  1. (6R)-L-erythro-5,6,7,8-tetrahydrobiopterin + L-tryptophan + O2 = 5-hydroxy-L-tryptophan + (4aS,6R)-4a-hydroxy-L-erythro-5,6,7,8-tetrahydrobiopterin
  2. (6R)-L-erythro-5,6,7,8-tetrahydrobiopterin + L-tryptophan + O2 = 5-hydroxy-L-tryptophan + (4aS,6R)-4a-hydroxy-L-erythro-5,6,7,8-tetrahydrobiopterin
  3. Transport of 3-Hydroxy-L-Kynurenine

  Catalysed by TPH1, TPH2, SLC7A5extracellular space, cytosolOne of 2 routes

• 5-aminolevulinic acidReach from here →
  1. (6R)-L-erythro-5,6,7,8-tetrahydrobiopterin + L-phenylalanine + O2 = (4aS,6R)-4a-hydroxy-L-erythro-5,6,7,8-tetrahydrobiopterin + L-tyrosine
  2. L-tyrosine + glyoxylate = 3-(4-hydroxyphenyl)pyruvate + glycine
  3. succinyl-CoA:glycine C-succinyltransferase (decarboxylating)

  Catalysed by PAH, AADAT, ALAS1, ALAS2 and 1 moremitochondrial matrix

• L-cysteineReach from here →
  1. (6R)-L-erythro-5,6,7,8-tetrahydrobiopterin + L-tryptophan + O2 = 5-hydroxy-L-tryptophan + (4aS,6R)-4a-hydroxy-L-erythro-5,6,7,8-tetrahydrobiopterin
  2. (6R)-L-erythro-5,6,7,8-tetrahydrobiopterin + L-tryptophan + O2 = 5-hydroxy-L-tryptophan + (4aS,6R)-4a-hydroxy-L-erythro-5,6,7,8-tetrahydrobiopterin
  3. transport of cysteine and tryptophan (cytosol to extracellular)

  Catalysed by TPH1, TPH2, SLC7A5extracellular space, cytosolOne of 14 routes

• indole-3-pyruvateReach from here →
  1. (6R)-L-erythro-5,6,7,8-tetrahydrobiopterin + L-tryptophan + O2 = 5-hydroxy-L-tryptophan + (4aS,6R)-4a-hydroxy-L-erythro-5,6,7,8-tetrahydrobiopterin
  2. (6R)-L-erythro-5,6,7,8-tetrahydrobiopterin + L-tryptophan + O2 = 5-hydroxy-L-tryptophan + (4aS,6R)-4a-hydroxy-L-erythro-5,6,7,8-tetrahydrobiopterin
  3. L-tryptophan + O2 + H2O = indole-3-pyruvate + H2O2 + NH4(+)

  Catalysed by TPH1, TPH2, IL4I1One of 2 routes

• 3,3'-diiodo-L-thyronine sulfateReach from here →
  1. (6R)-L-erythro-5,6,7,8-tetrahydrobiopterin(out) + a dicarboxylate(in) = (6R)-L-erythro-5,6,7,8-tetrahydrobiopterin(in) + a dicarboxylate(out)
  2. a dicarboxylate(in) + sulfate(out) = a dicarboxylate(out) + sulfate(in)
  3. Steryl-Sulfatase

  Catalysed by SLC22A6, SLC22A8, SLC25A35, SLC25A34 and 1 moreinner side of the membrane, outer side of the membrane, Golgi apparatusOne of 2 routes