5,6,7,8-tetrahydrobiopterin

Three steps

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

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

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

  Catalysed by PAH, SLC7A5, AGXT2cytosol, extracellular spaceOne of 2 routes

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

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

• (11Z)-octadecenoyl-CoAReach from here →
  1. L-phenylalanine,tetrahydrobiopterin:oxygen oxidoreductase(4-hydroxylating)
  2. Acetylation of Tyrosine
  3. Transport of 11-Octadecenoyl Carnitine into the Mitochondrial Matrix

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

• dodecanedioyl-CoAReach from here →
  1. L-phenylalanine,tetrahydrobiopterin:oxygen oxidoreductase(4-hydroxylating)
  2. Acetylation of Tyrosine
  3. Activation of dodecanedioic acid

  Catalysed by PAHcytosol, mitochondrial matrixOne of 4 routes

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

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

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

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

• hexanedioyl-CoAReach from here →
  1. L-phenylalanine,tetrahydrobiopterin:oxygen oxidoreductase(4-hydroxylating)
  2. Acetylation of Tyrosine
  3. Activation of adipic acid for Formation of Adipoyl Carnitine

  Catalysed by PAHcytosol, mitochondrial matrixOne of 4 routes

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

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

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

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

• (R)-3-hydroxy-(11Z)-octadecenoyl-CoAReach from here →
  1. L-phenylalanine,tetrahydrobiopterin:oxygen oxidoreductase(4-hydroxylating)
  2. Acetylation of Tyrosine
  3. Production of 3-Hydroxyoctadecenoylcarnitinec

  Catalysed by PAH, CPT1A, CPT1C, CPT1Bcytosol, mitochondrial matrix

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

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

• 4,8-dimethylnonanoyl-CoAReach from here →
  1. L-phenylalanine,tetrahydrobiopterin:oxygen oxidoreductase(4-hydroxylating)
  2. Acetylation of Tyrosine
  3. Carnitine Dimethyl Nonanoyl Transferase, Revsible, Peroxisomal

  Catalysed by PAH, CRAT, CROTcytosol, mitochondrial matrix, peroxisomeOne of 3 routes

• tetradecanedioyl-CoAReach from here →
  1. L-phenylalanine,tetrahydrobiopterin:oxygen oxidoreductase(4-hydroxylating)
  2. Acetylation of Tyrosine
  3. Fatty Acid Beta Oxidation (C16Dc->C14Dc), Peroxisomal

  Catalysed by PAH, ACAA1, HSD17B4, EHHADH and 1 morecytosol, mitochondrial matrix, peroxisome

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

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

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

  Catalysed by PAH, SLC27A2cytosol, mitochondrial matrixOne of 2 routes

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

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

• thio-Mo-molybdopterinReach from here →
  1. L-phenylalanine,tetrahydrobiopterin:oxygen oxidoreductase(4-hydroxylating)
  2. transport of cysteine 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

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

  Catalysed by PAH, SLC7A5cytosol, extracellular spaceOne of 2 routes

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

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

• (3E)-decenoyl-CoAReach from here →
  1. L-phenylalanine,tetrahydrobiopterin:oxygen oxidoreductase(4-hydroxylating)
  2. Acetylation of Tyrosine
  3. Fatty Acid Beta Oxidation (C12:1->C10:1), Mitochondrial

  Catalysed by PAH, ACADM, ECHS1, ACAA2 and 1 morecytosol, mitochondrial matrix

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

  Catalysed by PAH, SLC7A5, GLYATL2cytosol, extracellular space

• (2R,6)-dimethylheptanoyl-CoAReach from here →
  1. L-phenylalanine,tetrahydrobiopterin:oxygen oxidoreductase(4-hydroxylating)
  2. Acetylation of Tyrosine
  3. acetyl-CoA C-acyltransferase (3-oxo-4(R),8-dimethyl-nonanoyl-CoA)

  Catalysed by PAH, HADHA, ACAA2, HADHBcytosol, mitochondrial matrix

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

  Catalysed by PAH, SLC7A5, BAATcytosol, extracellular space

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

  Catalysed by PAH, SLC7A5, BAATcytosol, extracellular space

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

  Catalysed by PAH, SLC7A5, BAATcytosol, extracellular space

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

  Catalysed by PAH, SLC7A5, GLYATL1B, GLYATL1cytosol, extracellular space

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

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

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

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