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

Three steps

• L-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. (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 ornithine and tryptophan (cytosol to extracellular)

  Catalysed by TPH1, TPH2, SLC7A6extracellular space, cytosolOne of 9 routes

• N-methylserotoninReach 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. S-Adenosyl-L-methionine:amine N-methyltransferase

  Catalysed by TPH1, TPH2, DDC, INMTcytosol

• (5-hydroxyindol-3-yl)acetaldehydeReach 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. 5-Hydroxytryptamine:oxygen oxidoreductase(deaminating)(flavin-containing)

  Catalysed by TPH1, TPH2, DDC, MAOA and 1 morecytosolOne of 2 routes

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

  Catalysed by PAH, AADAT, GLDC, GCSHmitochondrial matrix

• benzoyl-CoAReach 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. N-benzoylglycine(out) + a dicarboxylate(in) = N-benzoylglycine(in) + a dicarboxylate(out)
  3. benzoyl-CoA + glycine = N-benzoylglycine + CoA + H(+)

  Catalysed by SLC22A6, SLC22A8, GLYATinner side of the membrane, outer side of the membrane

• 15-oxoprostaglandin E2Reach 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 E2(out) + a dicarboxylate(in) = prostaglandin E2(in) + a dicarboxylate(out)
  3. prostaglandin E2 + NAD(+) = 15-oxoprostaglandin E2 + NADH + H(+)

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

• prostaglandin H2Reach 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-F synthase (prostaglandin F2alpha)

  Catalysed by SLC22A6, SLC22A8inner side of the membrane, outer side of the membrane, cytosolOne of 4 routes

• prostaglandin D2Reach 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. aldo-keto reductase (prostaglandin D2)

  Catalysed by SLC22A6, SLC22A8, AKR1C3inner side of the membrane, outer side of the membrane, cytosol

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

  Catalysed by PAH, AADAT, PIPOXOne of 2 routes

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

  Catalysed by PAH, AADAT, SHMT1, SHMT2

• 2-(N(omega)-L-arginino)succinateReach 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-Citrulline:L-aspartate ligase (AMP-forming)

  Catalysed by PAH, SLC7A13, ASS1inner side of the membrane, outer side of the membrane, cytosolOne of 2 routes

• N(6)-(1,2-dicarboxyethyl)-AMPReach 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. IMP:L-aspartate ligase (GDP-forming)

  Catalysed by PAH, SLC7A13, ADSS2, ADSS1inner side of the membrane, outer side of the membrane, cytosolOne of 2 routes

• L-histidineReach 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-histidine(in) + L-phenylalanine(out) = L-histidine(out) + L-phenylalanine(in)

  Catalysed by TPH1, TPH2, PAH, SLC7A8inner 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-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. an acyl-CoA + glycine = an N-acylglycine + CoA + H(+)

  Catalysed by PAH, AADAT, GLYATL3, GLYAT and 1 more

• guanidinoacetateReach 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-Arginine:glycine amidinotransferase

  Catalysed by PAH, AADAT, GATMcytosolOne of 2 routes

• 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. (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 methionine and tryptophan (cytosol to extracellular)

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

• N-sulfo-D-glucosamineReach 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. N-sulfo-D-glucosamine + H2O = D-glucosamine + sulfate

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

• dehydroepiandrosterone 3-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. dehydroepiandrosterone 3-sulfate + H2O = 3beta-hydroxyandrost-5-en-17-one + sulfate + H(+)

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

• glutathioneReach 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-L-cysteine + glycine + ATP = glutathione + ADP + phosphate + H(+)

  Catalysed by PAH, AADAT, GSSOne of 2 routes

• 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. (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. Transport of L-Kynurenine

  Catalysed by TPH1, TPH2, PAH, SLC7A8extracellular space, cytosolOne of 2 routes

• beta-alanineReach 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 1-carboxy-lyase (beta-alanine-forming)

  Catalysed by PAH, SLC7A13, GAD2inner side of the membrane, outer side of the membrane, cytosolOne of 2 routes

• 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. (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 alanine and tryptophan (cytosol to extracellular)

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

• leukotriene C4Reach 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. leukotriene C4 hydrolysis

  Catalysed by PAH, AADATcytosol

• 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. (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 asparagine and tryptophan (cytosol to extracellular)

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

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

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

• adenosine 5'-phosphosulfateReach 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. ATP:sulfate adenylyltransferase

  Catalysed by SLC22A6, SLC22A8, SLC25A35, SLC25A34 and 2 moreinner side of the membrane, outer side of the membrane, cytosolOne of 4 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, AGXT2One of 2 routes

• (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-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. 1-(5-Phosphoribosyl)-5-amino-4-carboxyimidazole:L-aspartate ligase (ADP-forming)

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

• N-formyl-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. (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 = N-formyl-L-kynurenine

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

• N-(5Z,8Z,11Z,14Z)-eicosatetraenoyl-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. (5Z,8Z,11Z,14Z)-eicosatetraenoyl-CoA + glycine = N-(5Z,8Z,11Z,14Z)-eicosatetraenoyl-glycine + CoA + H(+)

  Catalysed by PAH, AADAT, GLYATL2One of 2 routes

• monodehydro-L-ascorbate radicalReach 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 + 2 L-ascorbate + O2 = (R)-noradrenaline + 2 monodehydro-L-ascorbate radical + H2O

  Catalysed by TH, DDC, DBH

• estrone 3-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. estrone 3-sulfate + H2O = estrone + sulfate + H(+)

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

• 3-sulfino-L-alanineReach 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. transport of 3-sulfinoalanine and aspartate (cytosol to mitochondria)

  Catalysed by PAH, SLC7A13, SLC25A12, SLC25A13inner side of the membrane, outer side of the membrane, mitochondrial matrix, cytosolOne of 4 routes

• (5-hydroxyindol-3-yl)acetateReach 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 5-Hydroxyindoleacetate

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

• (R)-noradrenalineReach 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, DBHcytosolOne of 2 routes

• methyl (indol-3-yl)acetateReach 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. (indol-3-yl)acetate(out) + a dicarboxylate(in) = (indol-3-yl)acetate(in) + a dicarboxylate(out)
  3. S-adenosyl-L-methionine:(indol-3-yl)acetate O-methyltransferase

  Catalysed by SLC22A6, SLC22A8, COMT, LRTOMTinner side of the membrane, outer side of the membrane, cytosol

• CHEBI:730073Reach 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

• CHEBI:742324Reach 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. S-Adenosyl-L-methionine:catechol O-methyltransferase

  Catalysed by TH, DDC, COMT, LRTOMTcytosol

• (2S)-2-aminobutanoateReach 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. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine

  Catalysed by PAH, AADAT, AGXT2

• glycodeoxycholateReach 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. Glycodeoxycholate amidohydrolase

  Catalysed by PAH, AADATcytosolOne of 2 routes

• N-tetradecanoylglycineReach 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. tetradecanoyl-CoA + glycine = N-tetradecanoylglycine + CoA + H(+)

  Catalysed by PAH, AADAT, GLYATL2

• N-eicosanoylglycinateReach 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. eicosanoyl-CoA + glycine = N-eicosanoylglycinate + CoA + H(+)

  Catalysed by PAH, AADAT, BAAT

• N-docosanoylglycineReach 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. docosanoyl-CoA + glycine = N-docosanoylglycine + CoA + H(+)

  Catalysed by PAH, AADAT, BAAT

• N-hexacosanoylglycineReach 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. hexacosanoyl-CoA + glycine = N-hexacosanoylglycine + CoA + H(+)

  Catalysed by PAH, AADAT, BAAT