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

Three steps

• isocyanateReach 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 citrulline and tryptophan (cytosol to extracellular)
  3. L-citrulline + H(+) = isocyanate + L-ornithine

  Catalysed by TPH1, TPH2, SLC7A9, SLC3A1 and 2 moreextracellular space, cytosol

• Asn-tRNA(Asn)Reach 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 asparagine and tryptophan (cytosol to extracellular)
  3. L-Asparagine:tRNA(Asn) ligase (AMP-forming)

  Catalysed by TPH1, TPH2, SLC7A5, NARS1extracellular space, cytosol

• glycocholateReach 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. bile acid-CoA: amino acid N-acyltransferase

  Catalysed by TPH1, TPH2, SLC7A5, BAATextracellular space, cytosol, peroxisomeOne of 3 routes

• hydrogen sulfideReach 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 hydrogen-sulfide-lyase (deaminating

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

• formic acidReach 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:oxygen 2,3-oxidoreductase (decyclizing)
  3. arylformamidase (L-formylkynurenine)

  Catalysed by TPH1, TPH2, IDO1, TDO2 and 2 morecytosolOne of 3 routes

• anthranilic 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. L-Kynurenine hydrolase

  Catalysed by PAH, SLC7A8, KYNUextracellular space, cytosol

• malonic acidReach 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. Malonate Transport, Antiport with Chloride

  Catalysed by TPH1, TPH2, SLC6A14extracellular space, cytosolOne of 2 routes

• (9Z)-octadecenoateReach 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-phenylalanine + (9Z)-octadecenoate = N-(9Z-octadecenoyl)-L-phenylalanine + H2O
  3. L-phenylalanine + (9Z)-octadecenoate = N-(9Z-octadecenoyl)-L-phenylalanine + H2O

  Catalysed by PAH, PM20D1

• (indol-3-yl)acetateReach 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. (indol-3-yl)acetate(out) + a dicarboxylate(in) = (indol-3-yl)acetate(in) + a dicarboxylate(out)

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

• (5Z,8Z,11Z,14Z)-eicosatetraenoateReach 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. (5Z,8Z,11Z,14Z)-eicosatetraenoate + L-phenylalanine = N-(5Z,8Z,11Z,14Z-eicosatetraenoyl)-L-phenylalanine + H2O
  3. (5Z,8Z,11Z,14Z)-eicosatetraenoate + L-phenylalanine = N-(5Z,8Z,11Z,14Z-eicosatetraenoyl)-L-phenylalanine + H2O

  Catalysed by PAH, PM20D1

• L-lysineReach 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 leucine and tryptophan (cytosol to extracellular)
  3. L-lysine(in) + L-leucine(out) + Na(+)(out) = L-lysine(out) + L-leucine(in) + Na(+)(in)

  Catalysed by TPH1, TPH2, SLC7A5, SLC7A7extracellular space, cytosol, inner side of the membrane, outer side of the membraneOne of 5 routes

• D-lysineReach 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. D-lysine(in) + L-ornithine(out) = D-lysine(out) + L-ornithine(in)

  Catalysed by TPH1, TPH2, SLC7A6, SLC25A2extracellular space, cytosol, inner side of the membrane, outer side of the membraneOne of 2 routes

• putrescineReach 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. Putrescine Export via Putrescine/Arginine Antiporter

  Catalysed by TPH1, TPH2, SLC7A6, SLC3A2 and 1 moreextracellular space, cytosolOne of 4 routes

• D-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 ornithine and tryptophan (cytosol to extracellular)
  3. D-arginine(in) + L-ornithine(out) = D-arginine(out) + L-ornithine(in)

  Catalysed by TPH1, TPH2, SLC7A6, SLC25A2extracellular space, cytosol, inner side of the membrane, outer side of the membraneOne of 2 routes

• N-carbamoyl-L-aspartateReach 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. carbamoyl phosphate + L-aspartate = N-carbamoyl-L-aspartate + phosphate + H(+)

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

• 4-methylthio-2-oxobutanoic acidReach 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. L-Methionine:oxygen oxidoreductase (deaminating)

  Catalysed by TPH1, TPH2, SLC7A5, IL4I1extracellular space, cytosol

• di-mu-sulfido-diiron(2+)Reach 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. De novo synthesis of 2Fe2S

  Catalysed by TPH1, TPH2, SLC7A5, NDUFAB1 and 6 moreextracellular space, cytosol, mitochondrial matrix

• serotoninReach 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, Tetrahydrobiopterin:Oxygen Oxidoreductase (5-Hydroxylating)
  3. 5-Hydroxy-L-tryptophan decarboxy-lyase

  Catalysed by TPH1, TPH2, DDCcytosolOne of 2 routes

• (S)-3-methyl-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 isoleucine and tryptophan (cytosol to extracellular)
  3. L-isoleucine + 2-oxoglutarate = (S)-3-methyl-2-oxopentanoate + L-glutamate

  Catalysed by TPH1, TPH2, SLC7A5, BCAT2 and 1 moreextracellular space, cytosol

• D-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 alanine and tryptophan (cytosol to extracellular)
  3. D-cysteine(out) + L-alanine(in) = D-cysteine(in) + L-alanine(out)

  Catalysed by TPH1, TPH2, SLC7A5, SLC7A10extracellular space, cytosol, inner side of the membrane, outer side of the membraneOne of 2 routes

• D-serineReach 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. D-serine(out) + L-alanine(in) = D-serine(in) + L-alanine(out)

  Catalysed by TPH1, TPH2, SLC7A5, SLC7A10extracellular space, cytosol, inner side of the membrane, outer side of the membraneOne of 10 routes

• 5-aminolevulinateReach 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. succinyl-CoA + glycine + H(+) = 5-aminolevulinate + CO2 + CoA

  Catalysed by TPH1, TPH2, SLC7A5, ALAS1 and 1 moreextracellular space, cytosol

• 3-methyl-2-oxovaleric acidReach 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 isoleucine and tryptophan (cytosol to extracellular)
  3. L-Leucine:2-oxoglutarate aminotransferase

  Catalysed by TPH1, TPH2, SLC7A5, BCAT1extracellular space, cytosolOne of 2 routes

• 3-(4-hydroxyphenyl)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. transport of tryptophan and tyrosine (cytosol to extracellular)
  3. L-tyrosine:2-oxoglutarate aminotransferase

  Catalysed by TPH1, TPH2, SLC7A5, GOT2 and 2 moreextracellular space, cytosolOne of 6 routes

• glycochenodeoxycholateReach 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. chenodeoxycholoyl-CoA + glycine = glycochenodeoxycholate + CoA + H(+)

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

• glycochenodeoxycholic acidReach 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. bile acid-CoA: amino acid N-acyltransferase

  Catalysed by TPH1, TPH2, SLC7A5, BAATextracellular space, cytosol, peroxisomeOne of 3 routes

• N-formylanthranilic acidReach 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:oxygen 2,3-oxidoreductase (decyclizing)
  3. Formylkynurenine hydrolase

  Catalysed by TPH1, TPH2, IDO1, TDO2 and 2 morecytosol

• glyoxylateReach 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. glycine + 2-oxoglutarate = glyoxylate + L-glutamate

  Catalysed by TPH1, TPH2, SLC7A5, AADATextracellular space, cytosolOne of 13 routes

• glycolithocholic acidReach 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. bile acid-CoA:amino acid N-acyltransferase (glycine)

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

• L-2-amino-3-oxobutanoic acidReach 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. Acetyl-CoA:glycine C-acetyltransferase

  Catalysed by TPH1, TPH2, SLC7A5, GCATextracellular space, cytosol, mitochondrial matrix

• L-erythro-7,8-dihydrobiopterinReach 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. L-erythro-7,8-dihydrobiopterin(out) + a dicarboxylate(in) = L-erythro-7,8-dihydrobiopterin(in) + a dicarboxylate(out)

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

• gamma-Glu-AlaReach 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, GGT1 and 5 moreextracellular space, cytosol

• S(8)-aminomethyldihydrolipoamideReach 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. dihydrolipoyl dehydrogenase ([protein]-N6-(lipoyl)lysine)

  Catalysed by TPH1, TPH2, SLC7A5, GLDC and 1 moreextracellular space, cytosol, mitochondrial matrix

• taurineReach 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. Neurotransmitter:Sodium Symporter (Nss) Tcdb:2.A.22.3.3

  Catalysed by TPH1, TPH2, SLC6A14, SLC6A6extracellular space, cytosolOne of 2 routes

• prostaglandin F2alphaReach 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 F2alpha(out) + a dicarboxylate(in) = prostaglandin F2alpha(in) + a dicarboxylate(out)

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

• D-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. D-alanine(out) + L-alanine(in) = D-alanine(in) + L-alanine(out)

  Catalysed by TPH1, TPH2, SLC7A5, SLC7A10extracellular space, cytosol, inner side of the membrane, outer side of the membraneOne of 2 routes

• sarcosineReach 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. sarcosine + O2 + H2O = formaldehyde + glycine + H2O2

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

• (6S)-5,6,7,8-tetrahydrofolateReach 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. (6R)-5,10-methylene-5,6,7,8-tetrahydrofolate + glycine + H2O = (6S)-5,6,7,8-tetrahydrofolate + L-serine

  Catalysed by TPH1, TPH2, SLC7A5, SHMT1 and 1 moreextracellular space, cytosol

• 2-(N(omega)-L-arginino)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. transport of arginine and tryptophan (cytosol to extracellular)
  3. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)

  Catalysed by TPH1, TPH2, SLC7A6, ASLextracellular space, cytosolOne of 6 routes

• carnosineReach 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-histidine(in) + L-phenylalanine(out) = L-histidine(out) + L-phenylalanine(in)
  3. beta-alanine + L-histidine + ATP = carnosine + ADP + phosphate + H(+)

  Catalysed by PAH, SLC7A8, CARNS1inner side of the membrane, outer side of the membrane

• 4-guanidinobutanoateReach 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. 4-aminobutanoate + L-arginine = 4-guanidinobutanoate + L-ornithine

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

• N(6)-(1,2-dicarboxyethyl)-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. L-tryptophan(out) + L-aspartate(in) = L-tryptophan(in) + L-aspartate(out)
  3. IMP:L-aspartate ligase (GDP-forming)

  Catalysed by TPH1, TPH2, SLC7A13, ADSS2 and 1 moreinner side of the membrane, outer side of the membrane, cytosolOne of 2 routes

• 3-guanidinopropanoateReach 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. beta-alanine + L-arginine = 3-guanidinopropanoate + L-ornithine

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

• D-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 ornithine and tryptophan (cytosol to extracellular)
  3. D-ornithine(in) + L-ornithine(out) = D-ornithine(out) + L-ornithine(in)

  Catalysed by TPH1, TPH2, SLC7A6, SLC25A2extracellular space, cytosol, inner side of the membrane, outer side of the membraneOne of 2 routes

• an N-acylglycineReach 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. an acyl-CoA + glycine = an N-acylglycine + CoA + H(+)

  Catalysed by TPH1, TPH2, SLC7A5, GLYATL3 and 2 moreextracellular space, cytosol

• 2-oxo-3-sulfanylpropanoateReach 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:2-oxoglutarate aminotransferase

  Catalysed by TPH1, TPH2, SLC7A5, GOT2 and 1 moreextracellular space, cytosolOne of 3 routes

• guanidinoacetateReach 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. L-Arginine:glycine amidinotransferase

  Catalysed by TPH1, TPH2, SLC7A5, GATMextracellular space, cytosolOne of 5 routes

• 3',5'-cyclic GMPReach 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 GMP(out) + a dicarboxylate(in) = 3',5'-cyclic GMP(in) + a dicarboxylate(out)

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

• D-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. D-threonine(out) + L-alanine(in) = D-threonine(in) + L-alanine(out)

  Catalysed by TPH1, TPH2, SLC7A5, SLC7A10extracellular space, cytosol, inner side of the membrane, outer side of the membraneOne of 2 routes

• L-dopaquinoneReach 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. L-Tyrosine,L-dopa:oxygen oxidoreductase

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