5,6,7,8-tetrahydrobiopterin

Three steps

• CHEBI:240943Reach from here →
  1. L-phenylalanine,tetrahydrobiopterin:oxygen oxidoreductase(4-hydroxylating)
  2. transport of glycine and tyrosine (cytosol to extracellular)
  3. glycine N-acyltransferase (2-methylbutyryl-CoA)

  Catalysed by PAH, SLC7A5, GLYAT, GLYATL2 and 1 morecytosol, extracellular space, mitochondrial matrixOne of 3 routes

• hypochlorous acidReach from here →
  1. L-phenylalanine,tetrahydrobiopterin:oxygen oxidoreductase(4-hydroxylating)
  2. Transport of L-Tyrosine by Atb0 Transporter
  3. Peroxidase

  Catalysed by PAH, SLC6A14, LPOcytosol, extracellular spaceOne of 4 routes

• potassium atomReach from here →
  1. L-Tryptophan, Tetrahydrobiopterin:Oxygen Oxidoreductase (5-Hydroxylating)
  2. 5-Hydroxy-L-tryptophan decarboxy-lyase
  3. transport of serotonin (cytosol to extracellular)

  Catalysed by TPH1, TPH2, DDC, SLC6A4cytosol, extracellular spaceOne of 3 routes

• phenylacetylglycineReach from here →
  1. L-phenylalanine,tetrahydrobiopterin:oxygen oxidoreductase(4-hydroxylating)
  2. transport of glycine and tyrosine (cytosol to extracellular)
  3. phenylacetyl-CoA:glycine N-phenylacetyltransferase

  Catalysed by PAH, SLC7A5, GLYATcytosol, extracellular spaceOne of 3 routes

• CHEBI:27540Reach from here →
  1. L-phenylalanine,tetrahydrobiopterin:oxygen oxidoreductase(4-hydroxylating)
  2. Acetylation of Tyrosine
  3. medium-chain acyl-CoA ligase (hexanoic acid)

  Catalysed by PAH, ACSM4, ACSM1, ACSM2A and 3 morecytosol, mitochondrial matrixOne of 6 routes

• 3,4-dihydroxyphenylacetaldehydeReach from here →
  1. L-Tyrosine,tetrahydrobiopterin:oxygen oxidoreductase (3-hydroxylating)
  2. 3,4-Dihydroxy-L-phenylalanine carboxy-lyase
  3. monoamine oxidase (dopamine)

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

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

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

• CHEBI:28666Reach from here →
  1. L-phenylalanine,tetrahydrobiopterin:oxygen oxidoreductase(4-hydroxylating)
  2. transport of glycine and tyrosine (cytosol to extracellular)
  3. LTD4 hydrolysis

  Catalysed by PAH, SLC7A5cytosol, extracellular space

• CHEBI:28701Reach from here →
  1. L-phenylalanine,tetrahydrobiopterin:oxygen oxidoreductase(4-hydroxylating)
  2. Acetylation of Tyrosine
  3. Propanoyl-CoA:acetyl-CoA C-acyltransferase

  Catalysed by PAH, ACAA1, SCP2, MYO5Bcytosol, mitochondrial matrix, peroxisomeOne of 7 routes

• pyridoxine 5'-phosphateReach from here →
  1. L-phenylalanine,tetrahydrobiopterin:oxygen oxidoreductase(4-hydroxylating)
  2. transport of alanine and tyrosine (cytosol to extracellular)
  3. L-selenocysteine selenide-lyase (L-alanine-forming)

  Catalysed by PAH, SLC7A5, SCLYcytosol, extracellular space

• N-acetyl-L-cysteineReach from here →
  1. L-phenylalanine,tetrahydrobiopterin:oxygen oxidoreductase(4-hydroxylating)
  2. transport of cysteine and tyrosine (cytosol to extracellular)
  3. N-acetylglutamate synthase (N-acetyl-L-cysteine)

  Catalysed by PAH, SLC7A5, NAGScytosol, extracellular space, mitochondrial matrix

• K(+)Reach from here →
  1. L-Tryptophan, Tetrahydrobiopterin:Oxygen Oxidoreductase (5-Hydroxylating)
  2. 5-Hydroxy-L-tryptophan decarboxy-lyase
  3. serotonin(out) + K(+)(in) + Na(+)(out) + H(+)(in) = serotonin(in) + K(+)(out) + Na(+)(in) + H(+)(out)

  Catalysed by TPH1, TPH2, DDC, SLC6A4cytosol, inner side of the membrane, outer side of the membraneOne of 3 routes

• Cys-tRNA(Cys)Reach from here →
  1. L-phenylalanine,tetrahydrobiopterin:oxygen oxidoreductase(4-hydroxylating)
  2. transport of cysteine and tyrosine (cytosol to extracellular)
  3. L-Cysteine:tRNA(Cys) ligase (AMP-forming)

  Catalysed by PAH, SLC7A5, CARS1cytosol, extracellular space

• Phe-tRNA(Phe)Reach from here →
  1. L-phenylalanine,tetrahydrobiopterin:oxygen oxidoreductase(4-hydroxylating)
  2. transport of phenylalanine and tyrosine (cytosol to extracellular)
  3. L-Phenylalanine:tRNA(Ala) ligase (AMP-forming)

  Catalysed by PAH, SLC7A5, FARSB, FARSAcytosol, extracellular space

• Gly-tRNA(Gly)Reach from here →
  1. L-phenylalanine,tetrahydrobiopterin:oxygen oxidoreductase(4-hydroxylating)
  2. transport of glycine and tyrosine (cytosol to extracellular)
  3. glycine:tRNA(Gly) ligase (AMP-forming)

  Catalysed by PAH, SLC7A5, GARS1cytosol, extracellular space

• Trp-tRNA(Trp)Reach from here →
  1. L-phenylalanine,tetrahydrobiopterin:oxygen oxidoreductase(4-hydroxylating)
  2. transport of tryptophan and tyrosine (cytosol to extracellular)
  3. L-Tryptophan -tRNA(Trp) ligase (AMP-forming)

  Catalysed by PAH, SLC7A5, WARS1cytosol, extracellular space

• Ile-tRNA(Ile)Reach from here →
  1. L-phenylalanine,tetrahydrobiopterin:oxygen oxidoreductase(4-hydroxylating)
  2. transport of isoleucine and tyrosine (cytosol to extracellular)
  3. L-Isoleucine:tRNA(Ile) ligase (AMP-forming)

  Catalysed by PAH, SLC7A5, IARS1cytosol, extracellular space

• Gln-tRNA(Gln)Reach from here →
  1. L-phenylalanine,tetrahydrobiopterin:oxygen oxidoreductase(4-hydroxylating)
  2. transport of glutamine and tyrosine (cytosol to extracellular)
  3. L-Glutamine:tRNA(Gln) ligase (AMP-forming)

  Catalysed by PAH, SLC7A5, QARS1cytosol, extracellular space

• isocyanateReach from here →
  1. L-phenylalanine,tetrahydrobiopterin:oxygen oxidoreductase(4-hydroxylating)
  2. transport of citrulline and tyrosine (cytosol to extracellular)
  3. L-citrulline + H(+) = isocyanate + L-ornithine

  Catalysed by PAH, SLC7A6, LACC1cytosol, extracellular space

• hypoioditeReach from here →
  1. L-phenylalanine,tetrahydrobiopterin:oxygen oxidoreductase(4-hydroxylating)
  2. thyroid peroxidase (iodine)
  3. iodide + H2O2 = hypoiodite + H2O

  Catalysed by PAH, TPO, MPO, LPOcytosol

• Asn-tRNA(Asn)Reach from here →
  1. L-phenylalanine,tetrahydrobiopterin:oxygen oxidoreductase(4-hydroxylating)
  2. transport of asparagine and tyrosine (cytosol to extracellular)
  3. L-Asparagine:tRNA(Asn) ligase (AMP-forming)

  Catalysed by PAH, SLC7A5, NARS1cytosol, extracellular space

• glycocholateReach from here →
  1. L-phenylalanine,tetrahydrobiopterin:oxygen oxidoreductase(4-hydroxylating)
  2. transport of glycine and tyrosine (cytosol to extracellular)
  3. bile acid-CoA: amino acid N-acyltransferase

  Catalysed by PAH, SLC7A5, BAATcytosol, extracellular space, peroxisomeOne of 3 routes

• hydrogen sulfideReach from here →
  1. L-phenylalanine,tetrahydrobiopterin:oxygen oxidoreductase(4-hydroxylating)
  2. transport of cysteine and tyrosine (cytosol to extracellular)
  3. L-cysteine hydrogen-sulfide-lyase (deaminating

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

• L-aspartateReach from here →
  1. L-Tyrosine,tetrahydrobiopterin:oxygen oxidoreductase (3-hydroxylating)
  2. solute carrier (L-dopa)
  3. L-leucine(out) + L-aspartate(in) = L-leucine(in) + L-aspartate(out)

  Catalysed by TH, SLC3A2, SLC7A5, SLC7A13cytosol, extracellular space, inner side of the membrane, outer side of the membraneOne of 18 routes

• CHEBI:30249Reach from here →
  1. L-phenylalanine,tetrahydrobiopterin:oxygen oxidoreductase(4-hydroxylating)
  2. transport of tryptophan and tyrosine (cytosol to extracellular)
  3. L-tryptophan:oxygen 2,3-oxidoreductase (decyclizing)

  Catalysed by PAH, SLC7A5, IDO1, TDO2 and 1 morecytosol, extracellular space

• formic acidReach from here →
  1. L-phenylalanine,tetrahydrobiopterin:oxygen oxidoreductase(4-hydroxylating)
  2. Transport of L-Tyrosine by Atb0 Transporter
  3. transport of chloride and formate (cytosol to extracellular)

  Catalysed by PAH, SLC6A14, SLC26A4cytosol, extracellular spaceOne of 2 routes

• malonic acidReach from here →
  1. L-phenylalanine,tetrahydrobiopterin:oxygen oxidoreductase(4-hydroxylating)
  2. Transport of L-Tyrosine by Atb0 Transporter
  3. Malonate Transport, Antiport with Chloride

  Catalysed by PAH, SLC6A14cytosol, extracellular spaceOne of 2 routes

• L-lysineReach from here →
  1. L-Tyrosine,tetrahydrobiopterin:oxygen oxidoreductase (3-hydroxylating)
  2. solute carrier (L-dopa)
  3. L-lysine(in) + L-leucine(out) + Na(+)(out) = L-lysine(out) + L-leucine(in) + Na(+)(in)

  Catalysed by TH, SLC3A2, SLC7A5, SLC7A7cytosol, extracellular space, inner side of the membrane, outer side of the membraneOne of 5 routes

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

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

• putrescineReach from here →
  1. L-phenylalanine,tetrahydrobiopterin:oxygen oxidoreductase(4-hydroxylating)
  2. transport of arginine and tyrosine (cytosol to extracellular)
  3. Putrescine Export via Putrescine/Arginine Antiporter

  Catalysed by PAH, SLC7A6, SLC3A2, SLC7A7cytosol, extracellular spaceOne of 4 routes

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

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

• 4-methylthio-2-oxobutanoic acidReach from here →
  1. L-phenylalanine,tetrahydrobiopterin:oxygen oxidoreductase(4-hydroxylating)
  2. transport of methionine and tyrosine (cytosol to extracellular)
  3. L-Methionine:oxygen oxidoreductase (deaminating)

  Catalysed by PAH, SLC7A5, IL4I1cytosol, extracellular space

• di-mu-sulfido-diiron(2+)Reach from here →
  1. L-phenylalanine,tetrahydrobiopterin:oxygen oxidoreductase(4-hydroxylating)
  2. transport of cysteine and tyrosine (cytosol to extracellular)
  3. De novo synthesis of 2Fe2S

  Catalysed by PAH, SLC7A5, NDUFAB1, FDXR and 5 morecytosol, extracellular space, mitochondrial matrix

• (S)-3-methyl-2-oxopentanoateReach from here →
  1. L-phenylalanine,tetrahydrobiopterin:oxygen oxidoreductase(4-hydroxylating)
  2. transport of isoleucine and tyrosine (cytosol to extracellular)
  3. L-isoleucine + 2-oxoglutarate = (S)-3-methyl-2-oxopentanoate + L-glutamate

  Catalysed by PAH, SLC7A5, BCAT2, BCAT1cytosol, extracellular space

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

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

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

  Catalysed by PAH, SLC7A5, SLC7A10cytosol, extracellular space, inner side of the membrane, outer side of the membraneOne of 10 routes

• 5-aminolevulinateReach from here →
  1. L-phenylalanine,tetrahydrobiopterin:oxygen oxidoreductase(4-hydroxylating)
  2. transport of glycine and tyrosine (cytosol to extracellular)
  3. succinyl-CoA + glycine + H(+) = 5-aminolevulinate + CO2 + CoA

  Catalysed by PAH, SLC7A5, ALAS1, ALAS2cytosol, extracellular space

• 3-methyl-2-oxovaleric acidReach from here →
  1. L-phenylalanine,tetrahydrobiopterin:oxygen oxidoreductase(4-hydroxylating)
  2. transport of isoleucine and tyrosine (cytosol to extracellular)
  3. L-Leucine:2-oxoglutarate aminotransferase

  Catalysed by PAH, SLC7A5, BCAT1cytosol, extracellular spaceOne of 2 routes

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

  Catalysed by PAH, SLC7A5, BAATcytosol, extracellular space

• glycochenodeoxycholic acidReach from here →
  1. L-phenylalanine,tetrahydrobiopterin:oxygen oxidoreductase(4-hydroxylating)
  2. transport of glycine and tyrosine (cytosol to extracellular)
  3. bile acid-CoA: amino acid N-acyltransferase

  Catalysed by PAH, SLC7A5, BAATcytosol, extracellular space, peroxisomeOne of 3 routes

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

  Catalysed by PAH, SLC7A5, AADATcytosol, extracellular spaceOne of 12 routes

• CHEBI:37946Reach from here →
  1. L-Tyrosine,tetrahydrobiopterin:oxygen oxidoreductase (3-hydroxylating)
  2. 3,4-Dihydroxy-L-phenylalanine carboxy-lyase
  3. aryl sulfotransferase (dopamine)

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

• glycolithocholic acidReach from here →
  1. L-phenylalanine,tetrahydrobiopterin:oxygen oxidoreductase(4-hydroxylating)
  2. transport of glycine and tyrosine (cytosol to extracellular)
  3. bile acid-CoA:amino acid N-acyltransferase (glycine)

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

• L-2-amino-3-oxobutanoic acidReach from here →
  1. L-phenylalanine,tetrahydrobiopterin:oxygen oxidoreductase(4-hydroxylating)
  2. transport of glycine and tyrosine (cytosol to extracellular)
  3. Acetyl-CoA:glycine C-acetyltransferase

  Catalysed by PAH, SLC7A5, GCATcytosol, extracellular space, mitochondrial matrix

• N-methylserotoninReach from here →
  1. L-Tryptophan, Tetrahydrobiopterin:Oxygen Oxidoreductase (5-Hydroxylating)
  2. 5-Hydroxy-L-tryptophan decarboxy-lyase
  3. S-Adenosyl-L-methionine:amine N-methyltransferase

  Catalysed by TPH1, TPH2, DDC, INMTcytosol

• (5-hydroxyindol-3-yl)acetaldehydeReach from here →
  1. L-Tryptophan, Tetrahydrobiopterin:Oxygen Oxidoreductase (5-Hydroxylating)
  2. 5-Hydroxy-L-tryptophan decarboxy-lyase
  3. 5-Hydroxytryptamine:oxygen oxidoreductase(deaminating)(flavin-containing)

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

• gamma-Glu-AlaReach from here →
  1. L-phenylalanine,tetrahydrobiopterin:oxygen oxidoreductase(4-hydroxylating)
  2. transport of alanine and tyrosine (cytosol to extracellular)
  3. gamma-glutamyltransferase (alanine)

  Catalysed by PAH, SLC7A5, GGT1, GGT5 and 4 morecytosol, extracellular space

• S(8)-aminomethyldihydrolipoamideReach from here →
  1. L-phenylalanine,tetrahydrobiopterin:oxygen oxidoreductase(4-hydroxylating)
  2. transport of glycine and tyrosine (cytosol to extracellular)
  3. dihydrolipoyl dehydrogenase ([protein]-N6-(lipoyl)lysine)

  Catalysed by PAH, SLC7A5, GLDC, GCSHcytosol, extracellular space, mitochondrial matrix

• taurineReach from here →
  1. L-phenylalanine,tetrahydrobiopterin:oxygen oxidoreductase(4-hydroxylating)
  2. Transport of L-Tyrosine by Atb0 Transporter
  3. Neurotransmitter:Sodium Symporter (Nss) Tcdb:2.A.22.3.3

  Catalysed by PAH, SLC6A14, SLC6A6cytosol, extracellular spaceOne of 4 routes

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

  Catalysed by PAH, TPOcytosolOne of 5 routes