5,6,7,8-tetrahydrobiopterin

Three steps

• (R)-methylmalonyl-CoAReach from here →
  1. L-phenylalanine,tetrahydrobiopterin:oxygen oxidoreductase(4-hydroxylating)
  2. Acetylation of Tyrosine
  3. aldehyde dehydrogenase (2-methyl-3-oxopropanoate)

  Catalysed by PAH, ALDH6A1cytosol, mitochondrial matrixOne of 2 routes

• (S)-methylmalonyl-CoAReach from here →
  1. L-phenylalanine,tetrahydrobiopterin:oxygen oxidoreductase(4-hydroxylating)
  2. Acetylation of Tyrosine
  3. (S)-methylmalonyl-CoA hydrolase

  Catalysed by PAHcytosol, mitochondrial matrix

• 3'-dephospho-CoAReach from here →
  1. L-phenylalanine,tetrahydrobiopterin:oxygen oxidoreductase(4-hydroxylating)
  2. Acetylation of Tyrosine
  3. acid phosphatase 2, lysosomal (CoA)

  Catalysed by PAH, ACP2cytosol, mitochondrial matrix, lysosomeOne of 2 routes

• (2E)-dodecenoyl-CoAReach from here →
  1. L-phenylalanine,tetrahydrobiopterin:oxygen oxidoreductase(4-hydroxylating)
  2. Acetylation of Tyrosine
  3. (2E)-dodecenoyl-CoA formation

  Catalysed by PAHcytosol, mitochondrial matrixOne of 3 routes

• 4,8,12-trimethyltridecanoyl-CoAReach from here →
  1. L-phenylalanine,tetrahydrobiopterin:oxygen oxidoreductase(4-hydroxylating)
  2. Acetylation of Tyrosine
  3. propanoyl-CoA C-acyltransferase (3-ketopristanoyl-CoA)

  Catalysed by PAH, ACAA1, MYO5Bcytosol, mitochondrial matrix, peroxisomeOne of 2 routes

• (6Z,9Z,12Z)-octadecatrienoyl-CoAReach from here →
  1. L-phenylalanine,tetrahydrobiopterin:oxygen oxidoreductase(4-hydroxylating)
  2. Acetylation of Tyrosine
  3. long-chain-fatty-acid-CoA ligase (gamma-linolenate)

  Catalysed by PAH, SLC27A2, ACSL4, ACSL3 and 5 morecytosol, mitochondrial matrixOne of 7 routes

• butanoyl-CoAReach from here →
  1. L-phenylalanine,tetrahydrobiopterin:oxygen oxidoreductase(4-hydroxylating)
  2. Acetylation of Tyrosine
  3. Butanoate:CoA ligase (AMP-forming)

  Catalysed by PAH, ACSM4, ACSM1, ACSM2A and 3 morecytosol, mitochondrial matrixOne of 9 routes

• dodecanoyl-CoAReach from here →
  1. L-phenylalanine,tetrahydrobiopterin:oxygen oxidoreductase(4-hydroxylating)
  2. Acetylation of Tyrosine
  3. long-chain-fatty-acid-CoA ligase (lauric acid)

  Catalysed by PAH, SLC27A2, ACSL4, ACSL3 and 5 morecytosol, mitochondrial matrixOne of 9 routes

• glutaryl-CoAReach from here →
  1. L-phenylalanine,tetrahydrobiopterin:oxygen oxidoreductase(4-hydroxylating)
  2. Acetylation of Tyrosine
  3. Production of Glutaryl Carnitine

  Catalysed by PAH, CPT1A, CPT1C, CPT1Bcytosol, mitochondrial matrixOne of 2 routes

• eicosanoyl-CoAReach from here →
  1. L-phenylalanine,tetrahydrobiopterin:oxygen oxidoreductase(4-hydroxylating)
  2. Acetylation of Tyrosine
  3. long-chain-fatty-acid-CoA ligase (eicosanoate)

  Catalysed by PAH, SLC27A2, ACSL4, ACSL3 and 5 morecytosol, mitochondrial matrixOne of 12 routes

• itaconyl-CoAReach from here →
  1. L-phenylalanine,tetrahydrobiopterin:oxygen oxidoreductase(4-hydroxylating)
  2. Acetylation of Tyrosine
  3. Itaconate:CoA ligase (GDP-forming)

  Catalysed by PAH, SUCLG1, SUCLG2, SUCLA2cytosol, mitochondrial matrixOne of 2 routes

• (9Z,12Z)-octadecadienoyl-CoAReach from here →
  1. L-phenylalanine,tetrahydrobiopterin:oxygen oxidoreductase(4-hydroxylating)
  2. Acetylation of Tyrosine
  3. long-chain-fatty-acid-CoA ligase ((6Z,9Z)-octadecadienoic acid)

  Catalysed by PAH, SLC27A2, ACSL4, ACSL3 and 5 morecytosol, mitochondrial matrixOne of 13 routes

• tetradecanoyl-CoAReach from here →
  1. L-phenylalanine,tetrahydrobiopterin:oxygen oxidoreductase(4-hydroxylating)
  2. Acetylation of Tyrosine
  3. long-chain-fatty-acid-CoA ligase (myristic acid)

  Catalysed by PAH, SLC27A2, ACSL4, ACSL3 and 5 morecytosol, mitochondrial matrixOne of 11 routes

• octanoyl-CoAReach from here →
  1. L-phenylalanine,tetrahydrobiopterin:oxygen oxidoreductase(4-hydroxylating)
  2. Acetylation of Tyrosine
  3. Octanoyl-CoA:L-carnitine O-octanoyltransferase

  Catalysed by PAH, CROTcytosol, mitochondrial matrixOne of 20 routes

• phytanoyl-CoAReach from here →
  1. L-phenylalanine,tetrahydrobiopterin:oxygen oxidoreductase(4-hydroxylating)
  2. Acetylation of Tyrosine
  3. phytanate-CoA ligase

  Catalysed by PAHcytosol, mitochondrial matrixOne of 5 routes

• octadecanoyl-CoAReach from here →
  1. L-phenylalanine,tetrahydrobiopterin:oxygen oxidoreductase(4-hydroxylating)
  2. Acetylation of Tyrosine
  3. long-chain-fatty-acid-CoA ligase (stearate)

  Catalysed by PAH, SLC27A2, ACSL4, ACSL3 and 4 morecytosol, mitochondrial matrixOne of 15 routes

• D-alanineReach from here →
  1. L-phenylalanine,tetrahydrobiopterin:oxygen oxidoreductase(4-hydroxylating)
  2. transport of alanine and tyrosine (cytosol to extracellular)
  3. D-alanine(out) + L-alanine(in) = D-alanine(in) + L-alanine(out)

  Catalysed by PAH, SLC7A5, SLC7A10cytosol, extracellular space, inner side of the membrane, outer side of the membraneOne of 2 routes

• sarcosineReach from here →
  1. L-phenylalanine,tetrahydrobiopterin:oxygen oxidoreductase(4-hydroxylating)
  2. transport of glycine and tyrosine (cytosol to extracellular)
  3. sarcosine + O2 + H2O = formaldehyde + glycine + H2O2

  Catalysed by PAH, SLC7A5, PIPOXcytosol, extracellular spaceOne of 2 routes

• (6S)-5,6,7,8-tetrahydrofolateReach from here →
  1. L-phenylalanine,tetrahydrobiopterin:oxygen oxidoreductase(4-hydroxylating)
  2. transport of glycine and tyrosine (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 PAH, SLC7A5, SHMT1, SHMT2cytosol, extracellular space

• 2-(N(omega)-L-arginino)succinateReach from here →
  1. L-phenylalanine,tetrahydrobiopterin:oxygen oxidoreductase(4-hydroxylating)
  2. transport of arginine and tyrosine (cytosol to extracellular)
  3. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)

  Catalysed by PAH, SLC7A6, ASLcytosol, extracellular spaceOne of 4 routes

• 4-guanidinobutanoateReach from here →
  1. L-phenylalanine,tetrahydrobiopterin:oxygen oxidoreductase(4-hydroxylating)
  2. transport of arginine and tyrosine (cytosol to extracellular)
  3. 4-aminobutanoate + L-arginine = 4-guanidinobutanoate + L-ornithine

  Catalysed by PAH, SLC7A6, GATMcytosol, extracellular space

• L-dopaReach from here →
  1. L-Tyrosine,tetrahydrobiopterin:oxygen oxidoreductase (3-hydroxylating)
  2. 3,4-Dihydroxy-L-phenylalanine carboxy-lyase
  3. L-dopa + H(+) = dopamine + CO2

  Catalysed by TH, DDCcytosolOne of 3 routes

• 3,5-diiodo-L-tyrosineReach from here →
  1. L-phenylalanine,tetrahydrobiopterin:oxygen oxidoreductase(4-hydroxylating)
  2. thyroid peroxidase (iodine)
  3. thyroid peroxidase (3-iodo-L-tyrosine)

  Catalysed by PAH, TPOcytosolOne of 2 routes

• 3-guanidinopropanoateReach from here →
  1. L-phenylalanine,tetrahydrobiopterin:oxygen oxidoreductase(4-hydroxylating)
  2. transport of arginine and tyrosine (cytosol to extracellular)
  3. beta-alanine + L-arginine = 3-guanidinopropanoate + L-ornithine

  Catalysed by PAH, SLC7A6, GATMcytosol, extracellular space

• L-histidineReach from here →
  1. L-phenylalanine,tetrahydrobiopterin:oxygen oxidoreductase(4-hydroxylating)
  2. transport of phenylalanine and tyrosine (cytosol to extracellular)
  3. L-histidine(in) + L-phenylalanine(out) = L-histidine(out) + L-phenylalanine(in)

  Catalysed by PAH, SLC7A5, SLC7A8cytosol, extracellular space, inner side of the membrane, outer side of the membraneOne of 7 routes

• D-ornithineReach from here →
  1. L-phenylalanine,tetrahydrobiopterin:oxygen oxidoreductase(4-hydroxylating)
  2. transport of ornithine and tyrosine (cytosol to extracellular)
  3. D-ornithine(in) + L-ornithine(out) = D-ornithine(out) + L-ornithine(in)

  Catalysed by PAH, SLC7A6, SLC25A2cytosol, extracellular space, inner side of the membrane, outer side of the membraneOne of 2 routes

• an N-acylglycineReach from here →
  1. L-phenylalanine,tetrahydrobiopterin:oxygen oxidoreductase(4-hydroxylating)
  2. transport of glycine and tyrosine (cytosol to extracellular)
  3. an acyl-CoA + glycine = an N-acylglycine + CoA + H(+)

  Catalysed by PAH, SLC7A5, GLYATL3, GLYAT and 1 morecytosol, extracellular space

• 2-oxo-3-sulfanylpropanoateReach from here →
  1. L-phenylalanine,tetrahydrobiopterin:oxygen oxidoreductase(4-hydroxylating)
  2. transport of cysteine and tyrosine (cytosol to extracellular)
  3. L-Cysteine:2-oxoglutarate aminotransferase

  Catalysed by PAH, SLC7A5, GOT2, GOT1cytosol, extracellular spaceOne of 3 routes

• guanidinoacetateReach from here →
  1. L-phenylalanine,tetrahydrobiopterin:oxygen oxidoreductase(4-hydroxylating)
  2. transport of glycine and tyrosine (cytosol to extracellular)
  3. L-Arginine:glycine amidinotransferase

  Catalysed by PAH, SLC7A5, GATMcytosol, extracellular spaceOne of 5 routes

• sphing-4-enineReach from here →
  1. L-phenylalanine,tetrahydrobiopterin:oxygen oxidoreductase(4-hydroxylating)
  2. Acetylation of Tyrosine
  3. acyl-CoA:sphingosine N-acyltransferase

  Catalysed by PAH, ASAH1, ACER2, ACER1 and 1 morecytosol, mitochondrial matrix, Golgi apparatusOne of 2 routes

• D-threonineReach from here →
  1. L-phenylalanine,tetrahydrobiopterin:oxygen oxidoreductase(4-hydroxylating)
  2. transport of alanine and tyrosine (cytosol to extracellular)
  3. D-threonine(out) + L-alanine(in) = D-threonine(in) + L-alanine(out)

  Catalysed by PAH, SLC7A5, SLC7A10cytosol, extracellular space, inner side of the membrane, outer side of the membraneOne of 2 routes

• spermidineReach from here →
  1. L-phenylalanine,tetrahydrobiopterin:oxygen oxidoreductase(4-hydroxylating)
  2. Acetylation of Tyrosine
  3. diamine N-acetyltransferase (N1-acetylspermidine)

  Catalysed by PAH, SAT1, SAT2cytosol, mitochondrial matrixOne of 3 routes

• glutathioneReach from here →
  1. L-phenylalanine,tetrahydrobiopterin:oxygen oxidoreductase(4-hydroxylating)
  2. transport of glycine and tyrosine (cytosol to extracellular)
  3. gamma-L-glutamyl-L-cysteine + glycine + ATP = glutathione + ADP + phosphate + H(+)

  Catalysed by PAH, SLC7A5, GSScytosol, extracellular space

• L-threonineReach from here →
  1. L-phenylalanine,tetrahydrobiopterin:oxygen oxidoreductase(4-hydroxylating)
  2. transport of alanine and tyrosine (cytosol to extracellular)
  3. L-threonine(out) + L-alanine(in) = L-threonine(in) + L-alanine(out)

  Catalysed by PAH, SLC7A5, SLC7A10cytosol, extracellular space, inner side of the membrane, outer side of the membraneOne of 8 routes

• L-saccharopineReach from here →
  1. L-phenylalanine,tetrahydrobiopterin:oxygen oxidoreductase(4-hydroxylating)
  2. transport of lysine and tyrosine (cytosol to extracellular)
  3. N6-(L-1,3-Dicarboxypropyl)-L-lysine:NADP+ oxidoreductase (L-lysine-forming)

  Catalysed by PAH, SLC7A6, AASScytosol, extracellular space, mitochondrial matrix

• L-kynurenineReach from here →
  1. L-Tyrosine,tetrahydrobiopterin:oxygen oxidoreductase (3-hydroxylating)
  2. solute carrier (L-dopa)
  3. Transport of L-Kynurenine

  Catalysed by TH, SLC3A2, SLC7A5, SLC43A1cytosol, extracellular spaceOne of 5 routes

• beta-alanineReach from here →
  1. L-phenylalanine,tetrahydrobiopterin:oxygen oxidoreductase(4-hydroxylating)
  2. transport of alanine and tyrosine (cytosol to extracellular)
  3. beta-alanine(out) + L-alanine(in) = beta-alanine(in) + L-alanine(out)

  Catalysed by PAH, SLC7A5, SLC7A10cytosol, extracellular space, inner side of the membrane, outer side of the membraneOne of 4 routes

• leukotriene C4Reach from here →
  1. L-phenylalanine,tetrahydrobiopterin:oxygen oxidoreductase(4-hydroxylating)
  2. transport of glycine and tyrosine (cytosol to extracellular)
  3. leukotriene C4 hydrolysis

  Catalysed by PAH, SLC7A5cytosol, extracellular space

• taurocyamineReach from here →
  1. L-phenylalanine,tetrahydrobiopterin:oxygen oxidoreductase(4-hydroxylating)
  2. transport of arginine and tyrosine (cytosol to extracellular)
  3. taurine + L-arginine = taurocyamine + L-ornithine

  Catalysed by PAH, SLC7A6, GATMcytosol, extracellular space

• L-glutamate 5-semialdehydeReach from here →
  1. L-phenylalanine,tetrahydrobiopterin:oxygen oxidoreductase(4-hydroxylating)
  2. transport of ornithine and tyrosine (cytosol to extracellular)
  3. L-ornithine + 2-oxoglutarate = L-glutamate 5-semialdehyde + L-glutamate

  Catalysed by PAH, SLC7A6, OATcytosol, extracellular space

• 3-hydroxy-L-kynurenineReach from here →
  1. L-phenylalanine,tetrahydrobiopterin:oxygen oxidoreductase(4-hydroxylating)
  2. transport of alanine and tyrosine (cytosol to extracellular)
  3. 3-hydroxy-L-kynurenine + H2O = 3-hydroxyanthranilate + L-alanine + H(+)

  Catalysed by PAH, SLC7A5, KYNUcytosol, extracellular space

• L,L-cystathionineReach from here →
  1. L-phenylalanine,tetrahydrobiopterin:oxygen oxidoreductase(4-hydroxylating)
  2. Acetylation of Tyrosine
  3. N-acetylglutamate synthase (N-acetyl-L-cystathionine)

  Catalysed by PAH, NAGScytosol, mitochondrial matrix

• gamma-L-glutamyl-L-cysteineReach from here →
  1. L-phenylalanine,tetrahydrobiopterin:oxygen oxidoreductase(4-hydroxylating)
  2. transport of cysteine and tyrosine (cytosol to extracellular)
  3. L-glutamate:L-cysteine gamma-ligase (ADP-forming)

  Catalysed by PAH, SLC7A5, GCLC, GCLMcytosol, extracellular spaceOne of 2 routes

• 6-amino-2-oxohexanoateReach from here →
  1. L-phenylalanine,tetrahydrobiopterin:oxygen oxidoreductase(4-hydroxylating)
  2. transport of lysine and tyrosine (cytosol to extracellular)
  3. L-lysine:oxygen 2-oxidoreductase (deaminating)

  Catalysed by PAH, SLC7A6cytosol, extracellular space, peroxisome

• L-homocysteineReach from here →
  1. L-phenylalanine,tetrahydrobiopterin:oxygen oxidoreductase(4-hydroxylating)
  2. transport of methionine and tyrosine (cytosol to extracellular)
  3. S-methyl-L-methionine + L-homocysteine = 2 L-methionine + H(+)

  Catalysed by PAH, SLC7A5, BHMT2cytosol, extracellular space

• carbamoyl phosphateReach from here →
  1. L-phenylalanine,tetrahydrobiopterin:oxygen oxidoreductase(4-hydroxylating)
  2. transport of glutamine and tyrosine (cytosol to extracellular)
  3. HCO3-:L-glutamine amido-ligase (ADP-forming, carbamate-phosphorylating)

  Catalysed by PAH, SLC7A5, CADcytosol, extracellular spaceOne of 4 routes

• S-methyl-L-methionineReach from here →
  1. L-phenylalanine,tetrahydrobiopterin:oxygen oxidoreductase(4-hydroxylating)
  2. transport of methionine and tyrosine (cytosol to extracellular)
  3. S-methyl-L-methionine + L-homocysteine = 2 L-methionine + H(+)

  Catalysed by PAH, SLC7A5, BHMT2cytosol, extracellular space

• L-tyrosineReach from here →
  1. L-phenylalanine,tetrahydrobiopterin:oxygen oxidoreductase(4-hydroxylating)
  2. transport of glycine and tyrosine (cytosol to extracellular)
  3. L-tyrosine + glyoxylate = 3-(4-hydroxyphenyl)pyruvate + glycine

  Catalysed by PAH, SLC7A5, AADATcytosol, extracellular spaceOne of 5 routes

• N(omega),N(omega)-dimethyl-L-arginineReach from here →
  1. L-phenylalanine,tetrahydrobiopterin:oxygen oxidoreductase(4-hydroxylating)
  2. transport of glycine and tyrosine (cytosol to extracellular)
  3. N(omega),N(omega)-dimethyl-L-arginine + glyoxylate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + glycine

  Catalysed by PAH, SLC7A5, AGXT2cytosol, extracellular spaceOne of 6 routes

• an acyl-CoAReach from here →
  1. L-phenylalanine,tetrahydrobiopterin:oxygen oxidoreductase(4-hydroxylating)
  2. Acetylation of Tyrosine
  3. acyl-CoA:sphingosine N-acyltransferase

  Catalysed by PAH, ASAH1, ACER2, ACER1 and 1 morecytosol, mitochondrial matrix, Golgi apparatusOne of 2 routes