5,6,7,8-tetrahydrobiopterin

Three steps

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

  Catalysed by PAH, SLC7A5, SLC1A4cytosol, extracellular space, inner side of the membrane, outer side of the membraneOne of 2 routes

• histamineReach from here →
  1. L-phenylalanine,tetrahydrobiopterin:oxygen oxidoreductase(4-hydroxylating)
  2. transport of histidine and tyrosine (cytosol to extracellular)
  3. L-histidine carboxy-lyase (histamine-forming)

  Catalysed by PAH, SLC7A6, HDCcytosol, extracellular space

• L-thyroxineReach from here →
  1. L-phenylalanine,tetrahydrobiopterin:oxygen oxidoreductase(4-hydroxylating)
  2. thyroid peroxidase (iodine)
  3. 3,3',5-triiodo-L-thyronine,iodide:acceptor oxidoreductase (iodinating)

  Catalysed by PAH, TPO, DIO1, DIO2cytosolOne of 2 routes

• 5-guanidino-2-oxopentanoateReach from here →
  1. L-phenylalanine,tetrahydrobiopterin:oxygen oxidoreductase(4-hydroxylating)
  2. transport of arginine and tyrosine (cytosol to extracellular)
  3. L-arginine + O2 + H2O = 5-guanidino-2-oxopentanoate + H2O2 + NH4(+)

  Catalysed by PAH, SLC7A6, IL4I1cytosol, extracellular space

• (3Z)-dodecenoyl-CoAReach from here →
  1. L-phenylalanine,tetrahydrobiopterin:oxygen oxidoreductase(4-hydroxylating)
  2. Acetylation of Tyrosine
  3. acetyl-CoA C-acyltransferase (3-oxomyrist-5-enoyl-CoA)

  Catalysed by PAH, HADHA, ACAA2, HADHBcytosol, mitochondrial matrixOne of 3 routes

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

  Catalysed by PAH, SLC7A5, CARS2cytosol, extracellular space

• N-formyl-L-kynurenineReach from here →
  1. L-phenylalanine,tetrahydrobiopterin:oxygen oxidoreductase(4-hydroxylating)
  2. transport of tryptophan and tyrosine (cytosol to extracellular)
  3. L-tryptophan + O2 = N-formyl-L-kynurenine

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

• (25R)-3alpha,7alpha,12alpha-trihydroxy-5beta-cholestan-26-oyl-CoAReach from here →
  1. L-phenylalanine,tetrahydrobiopterin:oxygen oxidoreductase(4-hydroxylating)
  2. Acetylation of Tyrosine
  3. 3alpha,7alpha,12alpha-trihydroxy-5beta-cholestanoate:CoA ligase (AMP-forming)

  Catalysed by PAH, SLC27A2, SLC27A5cytosol, mitochondrial matrix, endoplasmic reticulumOne of 3 routes

• 5-phospho-beta-D-ribosylamineReach from here →
  1. L-phenylalanine,tetrahydrobiopterin:oxygen oxidoreductase(4-hydroxylating)
  2. transport of glutamine and tyrosine (cytosol to extracellular)
  3. phosphoribosyl pyrophosphate amidotransferase (glutamine)

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

• D-glucosamine 6-phosphateReach from here →
  1. L-phenylalanine,tetrahydrobiopterin:oxygen oxidoreductase(4-hydroxylating)
  2. transport of glutamine and tyrosine (cytosol to extracellular)
  3. D-fructose 6-phosphate + L-glutamine = D-glucosamine 6-phosphate + L-glutamate

  Catalysed by PAH, SLC7A5, GFPT2, GFPT1cytosol, extracellular space

• L-methionine (S)-S-oxideReach from here →
  1. L-phenylalanine,tetrahydrobiopterin:oxygen oxidoreductase(4-hydroxylating)
  2. transport of methionine and tyrosine (cytosol to extracellular)
  3. [thioredoxin]-disulfide + L-methionine + H2O = L-methionine (S)-S-oxide + [thioredoxin]-dithiol

  Catalysed by PAH, SLC7A5, MSRAcytosol, extracellular space

• L-methionine (R)-S-oxideReach from here →
  1. L-phenylalanine,tetrahydrobiopterin:oxygen oxidoreductase(4-hydroxylating)
  2. transport of methionine and tyrosine (cytosol to extracellular)
  3. [thioredoxin]-disulfide + L-methionine + H2O = L-methionine (R)-S-oxide + [thioredoxin]-dithiol

  Catalysed by PAH, SLC7A5, MSRB3, MSRB1 and 1 morecytosol, extracellular space

• 5-amino-2-oxopentanoateReach from here →
  1. L-phenylalanine,tetrahydrobiopterin:oxygen oxidoreductase(4-hydroxylating)
  2. transport of ornithine and tyrosine (cytosol to extracellular)
  3. L-ornithine + pyruvate = 5-amino-2-oxopentanoate + L-alanine

  Catalysed by PAH, SLC7A6, AGXT2cytosol, extracellular spaceOne of 2 routes

• deoxycholoyl-CoAReach from here →
  1. L-phenylalanine,tetrahydrobiopterin:oxygen oxidoreductase(4-hydroxylating)
  2. Acetylation of Tyrosine
  3. cholate-CoA ligase (3alpha,12alpha-dihydroxy-5beta-cholanate)

  Catalysed by PAH, SLC27A2, SLC27A5cytosol, mitochondrial matrixOne of 4 routes

• N-(5Z,8Z,11Z,14Z)-eicosatetraenoyl-glycineReach from here →
  1. L-phenylalanine,tetrahydrobiopterin:oxygen oxidoreductase(4-hydroxylating)
  2. transport of glycine and tyrosine (cytosol to extracellular)
  3. (5Z,8Z,11Z,14Z)-eicosatetraenoyl-CoA + glycine = N-(5Z,8Z,11Z,14Z)-eicosatetraenoyl-glycine + CoA + H(+)

  Catalysed by PAH, SLC7A5, GLYATL2cytosol, extracellular spaceOne of 2 routes

• N-[(R)-4-phosphopantothenoyl]-L-cysteineReach from here →
  1. L-phenylalanine,tetrahydrobiopterin:oxygen oxidoreductase(4-hydroxylating)
  2. transport of cysteine and tyrosine (cytosol to extracellular)
  3. (R)-4'-phosphopantothenate:L-cysteine ligase

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

• monodehydro-L-ascorbate radicalReach from here →
  1. L-Tyrosine,tetrahydrobiopterin:oxygen oxidoreductase (3-hydroxylating)
  2. 3,4-Dihydroxy-L-phenylalanine carboxy-lyase
  3. dopamine + 2 L-ascorbate + O2 = (R)-noradrenaline + 2 monodehydro-L-ascorbate radical + H2O

  Catalysed by TH, DDC, DBHcytosol

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

  Catalysed by PAH, SLC6A14, SLC6A12cytosol, extracellular spaceOne of 4 routes

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

  Catalysed by PAH, SLC7A5, SLC1A4cytosol, extracellular space, inner side of the membrane, outer side of the membraneOne of 2 routes

• N-benzoylglycineReach from here →
  1. L-phenylalanine,tetrahydrobiopterin:oxygen oxidoreductase(4-hydroxylating)
  2. transport of glycine and tyrosine (cytosol to extracellular)
  3. Hippurate (Benzoylglycinate) Formation

  Catalysed by PAH, SLC7A5cytosol, extracellular space, mitochondrial matrixOne of 2 routes

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

  Catalysed by PAH, SLC7A5, CDO1cytosol, extracellular spaceOne of 2 routes

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

  Catalysed by PAH, ACSM4, ACSM1, ACSM2A and 3 morecytosol, mitochondrial matrixOne of 7 routes

• (2E)-hexadecenoyl-CoAReach from here →
  1. L-phenylalanine,tetrahydrobiopterin:oxygen oxidoreductase(4-hydroxylating)
  2. Acetylation of Tyrosine
  3. Carnitine O-Palmitoyltransferase

  Catalysed by PAH, CPT1A, CPT1C, CPT1Bcytosol, mitochondrial matrixOne of 4 routes

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

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

• L-cysteinylglycineReach 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, GGTLC3, GGT1 and 4 morecytosol, extracellular spaceOne of 2 routes

• 3-hydroxyisovaleryl-CoAReach from here →
  1. L-phenylalanine,tetrahydrobiopterin:oxygen oxidoreductase(4-hydroxylating)
  2. Acetylation of Tyrosine
  3. (3-Hydroxyisovalerylcoa->3-Hydroxyisovalerylcarnitine)

  Catalysed by PAH, CPT1A, CPT1Bcytosol, mitochondrial matrix

• CHEBI:63544Reach from here →
  1. L-phenylalanine,tetrahydrobiopterin:oxygen oxidoreductase(4-hydroxylating)
  2. Acetylation of Tyrosine
  3. long-chain-fatty-acid-CoA ligase (adrenic acid)

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

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

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

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

  Catalysed by PAH, SLC27A2, ACSL4, ACSL3 and 5 morecytosol, mitochondrial matrixOne of 10 routes

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

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

• N(5)-[(Z)-amino(hydroxyimino)methyl]ornithineReach from here →
  1. L-phenylalanine,tetrahydrobiopterin:oxygen oxidoreductase(4-hydroxylating)
  2. transport of arginine and tyrosine (cytosol to extracellular)
  3. L-arginine,NADPH:oxygen oxidoreductase (N-(omega)-hydroxyarginine-forming)

  Catalysed by PAH, SLC7A6, NOS3, NOS1 and 1 morecytosol, extracellular space

• Mo-molybdopterinReach from here →
  1. L-phenylalanine,tetrahydrobiopterin:oxygen oxidoreductase(4-hydroxylating)
  2. transport of alanine and tyrosine (cytosol to extracellular)
  3. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O

  Catalysed by PAH, SLC7A5, MOCOScytosol, extracellular space

• (2E)-octadecenoyl-CoAReach from here →
  1. L-phenylalanine,tetrahydrobiopterin:oxygen oxidoreductase(4-hydroxylating)
  2. Acetylation of Tyrosine
  3. Carnitine O-Palmitoyltransferase

  Catalysed by PAH, CPT1A, CPT1C, CPT1Bcytosol, mitochondrial matrixOne of 3 routes

• (6Z,9Z,12Z,15Z)-octadecatetraenoyl-CoAReach from here →
  1. L-phenylalanine,tetrahydrobiopterin:oxygen oxidoreductase(4-hydroxylating)
  2. Acetylation of Tyrosine
  3. long-chain-fatty-acid-CoA ligase (stearidonic acid)

  Catalysed by PAH, SLC27A2, ACSL4, ACSL3 and 5 morecytosol, mitochondrial matrixOne of 8 routes

• (R)-noradrenalineReach from here →
  1. L-Tyrosine,tetrahydrobiopterin:oxygen oxidoreductase (3-hydroxylating)
  2. 3,4-Dihydroxy-L-phenylalanine carboxy-lyase
  3. dopamine,ascorbate:oxygen oxidoreductase (beta-hydroxylating)

  Catalysed by TH, DDC, DBHcytosolOne of 2 routes

• (5Z,8Z,11Z,14Z,17Z)-eicosapentaenoyl-CoAReach from here →
  1. L-phenylalanine,tetrahydrobiopterin:oxygen oxidoreductase(4-hydroxylating)
  2. Acetylation of Tyrosine
  3. long-chain-fatty-acid-CoA ligase (EPA)

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

• (7Z,10Z,13Z,16Z,19Z)-docosapentaenoyl-CoAReach from here →
  1. L-phenylalanine,tetrahydrobiopterin:oxygen oxidoreductase(4-hydroxylating)
  2. Acetylation of Tyrosine
  3. long-chain-fatty-acid-CoA ligase (DPA)

  Catalysed by PAH, SLC27A2, ACSL4, ACSL3 and 5 morecytosol, mitochondrial matrixOne of 6 routes

• (9Z,12Z,15Z)-octadecatrienoyl-CoAReach from here →
  1. L-phenylalanine,tetrahydrobiopterin:oxygen oxidoreductase(4-hydroxylating)
  2. Acetylation of Tyrosine
  3. long-chain-fatty-acid-CoA ligase (linolenate)

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

• (13Z)-docosenoyl-CoAReach from here →
  1. L-phenylalanine,tetrahydrobiopterin:oxygen oxidoreductase(4-hydroxylating)
  2. Acetylation of Tyrosine
  3. long-chain-fatty-acid-CoA ligase (cis-erucic acid)

  Catalysed by PAH, SLC27A2, ACSL4, ACSL3 and 5 morecytosol, mitochondrial matrixOne of 6 routes

• (11Z)-eicosenoyl-CoAReach from here →
  1. L-phenylalanine,tetrahydrobiopterin:oxygen oxidoreductase(4-hydroxylating)
  2. Acetylation of Tyrosine
  3. long-chain-fatty-acid-CoA ligase (cis-gondoic acid)

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

• (9Z,12Z,15Z,18Z,21Z)-tetracosapentaenoyl-CoAReach from here →
  1. L-phenylalanine,tetrahydrobiopterin:oxygen oxidoreductase(4-hydroxylating)
  2. Acetylation of Tyrosine
  3. long-chain-fatty-acid-CoA ligase ((9Z,12Z,15Z,18Z,21Z)-TPA)

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

• (6Z,9Z,12Z,15Z,18Z,21Z)-tetracosahexaenoyl-CoAReach from here →
  1. L-phenylalanine,tetrahydrobiopterin:oxygen oxidoreductase(4-hydroxylating)
  2. Acetylation of Tyrosine
  3. long-chain-fatty-acid-CoA ligase ((6Z,9Z,12Z,15Z,18Z,21Z)-THA)

  Catalysed by PAH, SLC27A2, ACSL4, ACSL3 and 5 morecytosol, mitochondrial matrixOne of 4 routes

• (15Z)-tetracosenoyl-CoAReach from here →
  1. L-phenylalanine,tetrahydrobiopterin:oxygen oxidoreductase(4-hydroxylating)
  2. Acetylation of Tyrosine
  3. long-chain-fatty-acid-CoA ligase (nervonic acid)

  Catalysed by PAH, SLC27A2, ACSL4, ACSL3 and 5 morecytosol, mitochondrial matrixOne of 6 routes

• (17Z)-hexacosenoyl-CoAReach from here →
  1. L-phenylalanine,tetrahydrobiopterin:oxygen oxidoreductase(4-hydroxylating)
  2. Acetylation of Tyrosine
  3. long-chain-fatty-acid-CoA ligase (ximenic acid)

  Catalysed by PAH, SLC27A2, ACSL4, ACSL3 and 5 morecytosol, mitochondrial matrixOne of 4 routes

• CHEBI:742324Reach from here →
  1. L-Tyrosine,tetrahydrobiopterin:oxygen oxidoreductase (3-hydroxylating)
  2. 3,4-Dihydroxy-L-phenylalanine carboxy-lyase
  3. S-Adenosyl-L-methionine:catechol O-methyltransferase

  Catalysed by TH, DDC, COMT, LRTOMTcytosol

• (8Z,11Z,14Z)-eicosatrienoyl-CoAReach from here →
  1. L-phenylalanine,tetrahydrobiopterin:oxygen oxidoreductase(4-hydroxylating)
  2. Acetylation of Tyrosine
  3. long-chain-fatty-acid-CoA ligase (dihomo-gamma-linolenate)

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

• (8Z,11Z,14Z,17Z)-eicosatetraenoyl-CoAReach from here →
  1. L-phenylalanine,tetrahydrobiopterin:oxygen oxidoreductase(4-hydroxylating)
  2. Acetylation of Tyrosine
  3. long-chain-fatty-acid-CoA ligase (omega-3-arachidonic acid)

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

• (4Z,7Z,10Z,13Z,16Z,19Z)-docosahexaenoyl-CoAReach from here →
  1. L-phenylalanine,tetrahydrobiopterin:oxygen oxidoreductase(4-hydroxylating)
  2. Acetylation of Tyrosine
  3. long-chain-fatty-acid-CoA ligase (DHA)

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

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

  Catalysed by PAH, SLC27A2, ACSL4, ACSL3 and 5 morecytosol, mitochondrial matrixOne of 6 routes

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

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