(4aS,6R)-4a-hydroxy-L-erythro-5,6,7,8-tetrahydrobiopterin

Three steps

• glutathioneReach 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. transport of glycine and tryptophan (cytosol to extracellular)
  3. gamma-L-glutamyl-L-cysteine + glycine + ATP = glutathione + ADP + phosphate + H(+)

  Catalysed by TPH1, TPH2, SLC7A5, GSSextracellular space, cytosol

• L-threonineReach 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. transport of alanine and tryptophan (cytosol to extracellular)
  3. L-threonine(out) + L-alanine(in) = L-threonine(in) + L-alanine(out)

  Catalysed by TPH1, TPH2, SLC7A5, SLC7A10extracellular space, cytosol, inner side of the membrane, outer side of the membraneOne of 8 routes

• L-saccharopineReach 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. transport of lysine and tryptophan (cytosol to extracellular)
  3. N6-(L-1,3-Dicarboxypropyl)-L-lysine:NADP+ oxidoreductase (L-lysine-forming)

  Catalysed by TPH1, TPH2, SLC7A6, AASSextracellular space, cytosol, mitochondrial matrix

• beta-alanineReach 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. transport of alanine and tryptophan (cytosol to extracellular)
  3. beta-alanine(out) + L-alanine(in) = beta-alanine(in) + L-alanine(out)

  Catalysed by TPH1, TPH2, SLC7A5, SLC7A10extracellular space, cytosol, inner side of the membrane, outer side of the membraneOne of 6 routes

• leukotriene C4Reach 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. transport of glycine and tryptophan (cytosol to extracellular)
  3. leukotriene C4 hydrolysis

  Catalysed by TPH1, TPH2, SLC7A5extracellular space, cytosol

• taurocyamineReach 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. transport of arginine and tryptophan (cytosol to extracellular)
  3. taurine + L-arginine = taurocyamine + L-ornithine

  Catalysed by TPH1, TPH2, SLC7A6, GATMextracellular space, cytosol

• L-glutamate 5-semialdehydeReach 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. transport of ornithine and tryptophan (cytosol to extracellular)
  3. L-ornithine + 2-oxoglutarate = L-glutamate 5-semialdehyde + L-glutamate

  Catalysed by TPH1, TPH2, SLC7A6, OATextracellular space, cytosol

• N(4)-(beta-N-acetyl-D-glucosaminyl)-L-asparagineReach 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. L-tryptophan(out) + L-aspartate(in) = L-tryptophan(in) + L-aspartate(out)
  3. N(4)-(beta-N-acetyl-D-glucosaminyl)-L-asparagine + H2O = N-acetyl-beta-D-glucosaminylamine + L-aspartate + H(+)

  Catalysed by TPH1, TPH2, SLC7A13, AGAinner side of the membrane, outer side of the membrane

• 3-hydroxy-L-kynurenineReach 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. transport of alanine and tryptophan (cytosol to extracellular)
  3. 3-hydroxy-L-kynurenine + H2O = 3-hydroxyanthranilate + L-alanine + H(+)

  Catalysed by TPH1, TPH2, SLC7A5, KYNUextracellular space, cytosolOne of 2 routes

• 3',5'-cyclic AMPReach 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(out) + a dicarboxylate(in) = (6R)-L-erythro-5,6,7,8-tetrahydrobiopterin(in) + a dicarboxylate(out)
  3. 3',5'-cyclic AMP(out) + a dicarboxylate(in) = 3',5'-cyclic AMP(in) + a dicarboxylate(out)

  Catalysed by TPH1, TPH2, SLC22A6, SLC22A8inner side of the membrane, outer side of the membraneOne of 2 routes

• gamma-L-glutamyl-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. transport of cysteine and tryptophan (cytosol to extracellular)
  3. L-glutamate:L-cysteine gamma-ligase (ADP-forming)

  Catalysed by TPH1, TPH2, SLC7A5, GCLC and 1 moreextracellular space, cytosolOne of 2 routes

• 6-amino-2-oxohexanoateReach 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. transport of lysine and tryptophan (cytosol to extracellular)
  3. L-lysine:oxygen 2-oxidoreductase (deaminating)

  Catalysed by TPH1, TPH2, SLC7A6extracellular space, cytosol, peroxisome

• L-homocysteineReach 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. transport of methionine and tryptophan (cytosol to extracellular)
  3. S-methyl-L-methionine + L-homocysteine = 2 L-methionine + H(+)

  Catalysed by TPH1, TPH2, SLC7A5, BHMT2extracellular space, cytosol

• carbamoyl phosphateReach 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. transport of glutamine and tryptophan (cytosol to extracellular)
  3. HCO3-:L-glutamine amido-ligase (ADP-forming, carbamate-phosphorylating)

  Catalysed by TPH1, TPH2, SLC7A5, CADextracellular space, cytosolOne of 4 routes

• S-methyl-L-methionineReach 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. transport of methionine and tryptophan (cytosol to extracellular)
  3. S-methyl-L-methionine + L-homocysteine = 2 L-methionine + H(+)

  Catalysed by TPH1, TPH2, SLC7A5, BHMT2extracellular space, cytosol

• N(omega),N(omega)-dimethyl-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. transport of glycine and tryptophan (cytosol to extracellular)
  3. N(omega),N(omega)-dimethyl-L-arginine + glyoxylate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + glycine

  Catalysed by TPH1, TPH2, SLC7A5, AGXT2extracellular space, cytosolOne of 7 routes

• trans-4-hydroxy-L-prolineReach 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. transport of alanine and tryptophan (cytosol to extracellular)
  3. trans-4-hydroxy-L-proline(out) + L-alanine(in) + Na(+)(out) = trans-4-hydroxy-L-proline(in) + L-alanine(out) + Na(+)(in)

  Catalysed by TPH1, TPH2, SLC7A5, SLC1A4extracellular space, cytosol, inner side of the membrane, outer side of the membraneOne of 2 routes

• histamineReach 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. transport of histidine and tryptophan (cytosol to extracellular)
  3. L-histidine carboxy-lyase (histamine-forming)

  Catalysed by TPH1, TPH2, SLC7A6, HDCextracellular space, cytosolOne of 2 routes

• L-2-aminopentanoateReach 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. Transport of L-Kynurenine
  3. 2-oxopentanoate + L-kynurenine = L-2-aminopentanoate + kynurenate + H2O

  Catalysed by PAH, SLC7A8, AADATextracellular space, cytosol

• (2S)-2-[5-amino-1-(5-phospho-beta-D-ribosyl)imidazole-4-carboxamido]succinateReach 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. L-tryptophan(out) + L-aspartate(in) = L-tryptophan(in) + L-aspartate(out)
  3. 1-(5-Phosphoribosyl)-5-amino-4-carboxyimidazole:L-aspartate ligase (ADP-forming)

  Catalysed by TPH1, TPH2, SLC7A13, PAICSinner side of the membrane, outer side of the membrane, cytosolOne of 2 routes

• kynurenateReach 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. L-tryptophan + O2 + H2O = indole-3-pyruvate + H2O2 + NH4(+)
  3. indole-3-pyruvate + L-kynurenine = kynurenate + L-tryptophan + H2O

  Catalysed by TPH1, TPH2, IL4I1, AADATOne of 17 routes

• L-2-aminohexanoateReach 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. Transport of L-Kynurenine
  3. 2-oxohexanoate + L-kynurenine = L-2-aminohexanoate + kynurenate + H2O

  Catalysed by PAH, SLC7A8, AADATextracellular space, cytosol

• 5-guanidino-2-oxopentanoateReach 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. transport of arginine and tryptophan (cytosol to extracellular)
  3. L-arginine + O2 + H2O = 5-guanidino-2-oxopentanoate + H2O2 + NH4(+)

  Catalysed by TPH1, TPH2, SLC7A6, IL4I1extracellular space, cytosol

• S-sulfanyl-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. transport of cysteine and tryptophan (cytosol to extracellular)
  3. 2 L-cysteine = S-sulfanyl-L-cysteine + L-alanine

  Catalysed by TPH1, TPH2, SLC7A5, CARS2extracellular space, cytosol

• L-2-aminoadipateReach 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. Transport of L-Kynurenine
  3. 2-oxoadipate + L-kynurenine = L-2-aminoadipate + kynurenate + H2O

  Catalysed by PAH, SLC7A8, AADATextracellular space, cytosol

• 5-phospho-beta-D-ribosylamineReach 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. transport of glutamine and tryptophan (cytosol to extracellular)
  3. phosphoribosyl pyrophosphate amidotransferase (glutamine)

  Catalysed by TPH1, TPH2, SLC7A5, PPATextracellular space, cytosolOne of 2 routes

• D-glucosamine 6-phosphateReach 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. transport of glutamine and tryptophan (cytosol to extracellular)
  3. D-fructose 6-phosphate + L-glutamine = D-glucosamine 6-phosphate + L-glutamate

  Catalysed by TPH1, TPH2, SLC7A5, GFPT2 and 1 moreextracellular space, cytosol

• L-methionine (S)-S-oxideReach 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. transport of methionine and tryptophan (cytosol to extracellular)
  3. [thioredoxin]-disulfide + L-methionine + H2O = L-methionine (S)-S-oxide + [thioredoxin]-dithiol

  Catalysed by TPH1, TPH2, SLC7A5, MSRAextracellular space, cytosol

• L-methionine (R)-S-oxideReach 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. transport of methionine and tryptophan (cytosol to extracellular)
  3. [thioredoxin]-disulfide + L-methionine + H2O = L-methionine (R)-S-oxide + [thioredoxin]-dithiol

  Catalysed by TPH1, TPH2, SLC7A5, MSRB3 and 2 moreextracellular space, cytosol

• 5-amino-2-oxopentanoateReach 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. transport of ornithine and tryptophan (cytosol to extracellular)
  3. L-ornithine + pyruvate = 5-amino-2-oxopentanoate + L-alanine

  Catalysed by TPH1, TPH2, SLC7A6, AGXT2extracellular space, cytosolOne of 2 routes

• N-(5Z,8Z,11Z,14Z)-eicosatetraenoyl-glycineReach 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. transport of glycine and tryptophan (cytosol to extracellular)
  3. (5Z,8Z,11Z,14Z)-eicosatetraenoyl-CoA + glycine = N-(5Z,8Z,11Z,14Z)-eicosatetraenoyl-glycine + CoA + H(+)

  Catalysed by TPH1, TPH2, SLC7A5, GLYATL2extracellular space, cytosolOne of 2 routes

• N-[(R)-4-phosphopantothenoyl]-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. transport of cysteine and tryptophan (cytosol to extracellular)
  3. (R)-4'-phosphopantothenate:L-cysteine ligase

  Catalysed by TPH1, TPH2, SLC7A5, PPCSextracellular space, cytosolOne of 4 routes

• an L-alpha-amino 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. Transport of L-Kynurenine
  3. an L-alpha-amino acid(in) + L-kynurenine(out) = an L-alpha-amino acid(out) + L-kynurenine(in)

  Catalysed by PAH, SLC7A8, SLC7A11extracellular space, cytosol, inner side of the membrane, outer side of the membraneOne of 2 routes

• 4-aminobutanoateReach 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. Transport of L-Tryptophan by Atb0 Transporter
  3. transport of 4-aminobutyrate and chloride (cytosol to extracellular)

  Catalysed by TPH1, TPH2, SLC6A14, SLC6A12extracellular space, cytosolOne of 4 routes

• 3-iodo-L-tyrosineReach 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. transport of tryptophan and tyrosine (cytosol to extracellular)
  3. thyroid peroxidase (iodine)

  Catalysed by TPH1, TPH2, SLC7A5, TPOextracellular space, cytosolOne of 2 routes

• dopamineReach 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-tyrosine + O2 = (4aS,6R)-4a-hydroxy-L-erythro-5,6,7,8-tetrahydrobiopterin + L-dopa
  3. L-dopa + H(+) = dopamine + CO2

  Catalysed by TPH1, TPH2, TH, DDC

• L-prolineReach 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. transport of alanine and tryptophan (cytosol to extracellular)
  3. L-proline(out) + L-alanine(in) + Na(+)(out) = L-proline(in) + L-alanine(out) + Na(+)(in)

  Catalysed by TPH1, TPH2, SLC7A5, SLC1A4extracellular space, cytosol, inner side of the membrane, outer side of the membraneOne of 2 routes

• prostaglandin E2Reach 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(out) + a dicarboxylate(in) = (6R)-L-erythro-5,6,7,8-tetrahydrobiopterin(in) + a dicarboxylate(out)
  3. prostaglandin E2(out) + a dicarboxylate(in) = prostaglandin E2(in) + a dicarboxylate(out)

  Catalysed by TPH1, TPH2, SLC22A6, SLC22A8inner side of the membrane, outer side of the membraneOne of 2 routes

• N-benzoylglycineReach 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. transport of glycine and tryptophan (cytosol to extracellular)
  3. Hippurate (Benzoylglycinate) Formation

  Catalysed by TPH1, TPH2, SLC7A5extracellular space, cytosol, mitochondrial matrixOne of 4 routes

• 3-sulfino-L-alanineReach 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. transport of cysteine and tryptophan (cytosol to extracellular)
  3. L-Cysteine:oxygen oxidoreductase

  Catalysed by TPH1, TPH2, SLC7A5, CDO1extracellular space, cytosolOne of 6 routes

• L-cysteinylglycineReach 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. transport of alanine and tryptophan (cytosol to extracellular)
  3. gamma-glutamyltransferase (alanine)

  Catalysed by TPH1, TPH2, SLC7A5, GGTLC3 and 5 moreextracellular space, cytosolOne of 2 routes

• N(5)-[(Z)-amino(hydroxyimino)methyl]ornithineReach 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. transport of arginine and tryptophan (cytosol to extracellular)
  3. L-arginine,NADPH:oxygen oxidoreductase (N-(omega)-hydroxyarginine-forming)

  Catalysed by TPH1, TPH2, SLC7A6, NOS3 and 2 moreextracellular space, cytosol

• xanthurenateReach 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. Transport of 3-Hydroxy-L-Kynurenine
  3. Kynurenine-Oxoglutarate Transaminase

  Catalysed by TPH1, TPH2, SLC7A5, AADATextracellular space, cytosol, mitochondrial matrixOne of 2 routes

• Mo-molybdopterinReach 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. transport of alanine and tryptophan (cytosol to extracellular)
  3. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O

  Catalysed by TPH1, TPH2, SLC7A5, MOCOSextracellular space, cytosol

• (2S)-2-aminobutanoateReach 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. transport of glycine and tryptophan (cytosol to extracellular)
  3. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine

  Catalysed by TPH1, TPH2, SLC7A5, AGXT2extracellular space, cytosolOne of 3 routes

• 3-phenyllactateReach 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. Phenylalanine Transaminase, Mitochondrial
  3. Formation of Phenyl-Lactate

  Catalysed by PAH, GOT2, TATmitochondrial matrix, cytosol

• thio-Mo-molybdopterinReach 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. transport of cysteine and tryptophan (cytosol to extracellular)
  3. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O

  Catalysed by TPH1, TPH2, SLC7A5, MOCOSextracellular space, cytosol

• glycodeoxycholateReach 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. transport of glycine and tryptophan (cytosol to extracellular)
  3. Glycodeoxycholate amidohydrolase

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

• N-tetradecanoylglycineReach 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. transport of glycine and tryptophan (cytosol to extracellular)
  3. tetradecanoyl-CoA + glycine = N-tetradecanoylglycine + CoA + H(+)

  Catalysed by TPH1, TPH2, SLC7A5, GLYATL2extracellular space, cytosol

• N-eicosanoylglycinateReach 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. transport of glycine and tryptophan (cytosol to extracellular)
  3. eicosanoyl-CoA + glycine = N-eicosanoylglycinate + CoA + H(+)

  Catalysed by TPH1, TPH2, SLC7A5, BAATextracellular space, cytosol