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

Three steps

• N-acetylserotoninReach 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. acetyl-CoA:aralkylamine N-acetyltransferase

  Catalysed by TPH1, TPH2, DDC, AANATcytosol

• xanthineReach 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. xanthine + NAD(+) + H2O = urate + NADH + H(+)

  Catalysed by SLC22A6, SLC22A8, XDHinner side of the membrane, outer side of the membraneOne of 2 routes

• CHEBI:17895Reach 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 tyrosine (cytosol to extracellular)

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

• 3-phenylpyruvateReach 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. Phenylalanine Transaminase, Mitochondrial

  Catalysed by TPH1, TPH2, PAH, GOT2 and 1 moremitochondrial matrixOne of 5 routes

• fumaric 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 fumarate 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 2 routes

• CHEBI:18019Reach 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 lysine and tryptophan (cytosol to extracellular)

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

• L-glutamineReach 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 glutamine and tryptophan (cytosol to extracellular)

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

• 5-hydroxyisourateReach 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. urate:oxygen oxidoreductase

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

• N(1)-(5-phospho-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-D-ribosylamine:glycine ligase (ADP-forming)

  Catalysed by PAH, AADAT, GARTcytosol

• 2-phenylethylamineReach 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 carboxy-lyase (phenylethylamine-forming)

  Catalysed by TPH1, TPH2, PAH, DDCcytosol

• ophthalmateReach 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. gamma-L-glutamyl-(2S)-2-aminobutanoate + glycine + ATP = ophthalmate + ADP + phosphate + H(+)

  Catalysed by PAH, AADAT, GSS

• S-methylmercury-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-phenylalanine + O2 = (4aS,6R)-4a-hydroxy-L-erythro-5,6,7,8-tetrahydrobiopterin + L-tyrosine
  3. S-methylmercury-L-cysteine(in) + L-phenylalanine(out) = S-methylmercury-L-cysteine(out) + L-phenylalanine(in)

  Catalysed by TPH1, TPH2, PAH, SLC7A8inner side of the membrane, outer side of the membraneOne of 2 routes

• N(omega),N('omega)-dimethyl-L-arginineReach 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. N(omega),N('omega)-dimethyl-L-arginine + glyoxylate = 5-(3,3'-dimethylguanidino)-2-oxopentanoate + glycine

  Catalysed by PAH, AADAT, AGXT2

• 5,6,7,8-tetrahydrofolic 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. 5,10-Methylenetetrahydrofolate:glycine hydroxymethyltransferase

  Catalysed by PAH, AADAT, SHMT1cytosolOne of 2 routes

• serotonin O-sulfateReach 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. serotonin + 3'-phosphoadenylyl sulfate = serotonin O-sulfate + adenosine 3',5'-bisphosphate + H(+)

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

• CHEBI:240943Reach 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 N-acyltransferase (2-methylbutyryl-CoA)

  Catalysed by PAH, AADAT, GLYAT, GLYATL2 and 1 moremitochondrial matrix

• potassium atomReach 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 serotonin (cytosol to extracellular)

  Catalysed by TPH1, TPH2, DDC, SLC6A4extracellular space, cytosolOne of 2 routes

• folic 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. Folate Transport by Oats

  Catalysed by SLC22A6, SLC22A8, SLC25A35, SLC25A34 and 1 moreinner side of the membrane, outer side of the membrane, extracellular space, cytosolOne of 2 routes

• phenylacetylglycineReach 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. phenylacetyl-CoA:glycine N-phenylacetyltransferase

  Catalysed by PAH, AADAT, GLYATcytosolOne of 3 routes

• 3,4-dihydroxyphenylacetaldehydeReach 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. monoamine oxidase (dopamine)

  Catalysed by TH, DDC, AOC2, AOC1 and 3 morecytosolOne of 2 routes

• 5-hydroxytryptophanReach 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:28666Reach 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. LTD4 hydrolysis

  Catalysed by PAH, AADATcytosol

• K(+)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. 5-hydroxy-L-tryptophan + H(+) = serotonin + CO2
  3. serotonin(out) + K(+)(in) + Na(+)(out) + H(+)(in) = serotonin(in) + K(+)(out) + Na(+)(in) + H(+)(out)

  Catalysed by TPH1, TPH2, DDC, SLC6A4inner side of the membrane, outer side of the membraneOne of 3 routes

• Phe-tRNA(Phe)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. (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:tRNA(Ala) ligase (AMP-forming)

  Catalysed by TPH1, TPH2, PAH, FARSB and 1 morecytosol

• Gly-tRNA(Gly)Reach 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:tRNA(Gly) ligase (AMP-forming)

  Catalysed by PAH, AADAT, GARS1cytosol

• Asp-tRNA(Asp)Reach 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:tRNA(Asp) ligase (AMP-forming)

  Catalysed by PAH, SLC7A13, DARS1inner side of the membrane, outer side of the membrane, cytosol

• Trp-tRNA(Trp)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. (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 -tRNA(Trp) ligase (AMP-forming)

  Catalysed by TPH1, TPH2, WARS1cytosol

• chlorine(.)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. (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 L-Tryptophan by Atb0 Transporter

  Catalysed by TPH1, TPH2, SLC6A14extracellular space, cytosolOne of 8 routes

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

  Catalysed by PAH, AADAT, BAATperoxisomeOne of 3 routes

• D-aspartateReach 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. D-aspartate(in) + sulfate(out) = D-aspartate(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:30249Reach 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:oxygen 2,3-oxidoreductase (decyclizing)

  Catalysed by TPH1, TPH2, IDO1, TDO2 and 1 morecytosol

• oxalateReach 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. oxalate(in) + sulfate(out) = oxalate(out) + sulfate(in)

  Catalysed by SLC22A6, SLC22A8, SLC25A35, SLC25A34 and 5 moreinner side of the membrane, outer side of the membraneOne of 2 routes

• maleateReach 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. maleate(in) + sulfate(out) = maleate(out) + sulfate(in)

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

• malonic 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 malonate 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 2 routes

• glutarateReach 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. kynurenate(out) + a dicarboxylate(in) = kynurenate(in) + a dicarboxylate(out)
  3. kynurenate(out) + glutarate(in) = kynurenate(in) + glutarate(out)

  Catalysed by SLC22A6, SLC22A8inner side of the membrane, outer side of the membraneOne of 8 routes

• (2R,3R)-tartrateReach 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. (2R,3R)-tartrate(in) + sulfate(out) = (2R,3R)-tartrate(out) + sulfate(in)

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

• (S,S)-tartrateReach 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. (S,S)-tartrate(in) + sulfate(out) = (S,S)-tartrate(out) + sulfate(in)

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

• (3R)-citramalateReach 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. (3R)-citramalate(in) + sulfate(out) = (3R)-citramalate(out) + sulfate(in)

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

• (3S)-citramalateReach 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. (3S)-citramalate(in) + sulfate(out) = (3S)-citramalate(out) + sulfate(in)

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

• nicotinateReach 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. nicotinate(in) + urate(out) = nicotinate(out) + urate(in)

  Catalysed by SLC22A6, SLC22A8, SLC22A12, SLC22A13inner side of the membrane, outer side of the membrane

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

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

• thiosulfateReach 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. thiosulfate(in) + sulfate(out) = thiosulfate(out) + sulfate(in)

  Catalysed by SLC22A6, SLC22A8, SLC25A35, SLC25A34 and 4 moreinner side of the membrane, outer side of the membraneOne of 2 routes

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

  Catalysed by PAH, AADAT, ALAS1, ALAS2

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

  Catalysed by PAH, AADAT, BAAT

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

  Catalysed by PAH, AADAT, BAATperoxisomeOne of 3 routes

• glyoxylateReach 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. L-tyrosine + glyoxylate = 3-(4-hydroxyphenyl)pyruvate + glycine

  Catalysed by PAH, TAT, AADATOne of 12 routes

• CHEBI:37946Reach 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. aryl sulfotransferase (dopamine)

  Catalysed by TH, DDC, SULT1A3, SULT1A4 and 2 morecytosol

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

  Catalysed by PAH, AADAT, BAATcytosolOne of 2 routes

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

  Catalysed by PAH, AADAT, GCATmitochondrial matrix

• (S)-lactic 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. urate(out) + a dicarboxylate(in) = urate(in) + a dicarboxylate(out)
  3. transport of L-lactate and urate (cytosol to extracellular)

  Catalysed by SLC22A6, SLC22A8, SLC22A12inner side of the membrane, outer side of the membrane, extracellular space, cytosol