3-phenylpyruvate

Three steps

• 3-hydroxy-3-methylglutarateReach from here →
  1. 3-phenylpyruvate + L-kynurenine = kynurenate + L-phenylalanine + H2O
  2. kynurenate(out) + glutarate(in) = kynurenate(in) + glutarate(out)
  3. 3-hydroxy-3-methylglutarate + glutaryl-CoA = (3S)-3-hydroxy-3-methylglutaryl-CoA + glutarate

  Catalysed by AADAT, SLC22A6, SUGCTinner side of the membrane, outer side of the membrane

• sulfiteReach from here →
  1. glyoxylate + L-phenylalanine = 3-phenylpyruvate + glycine
  2. glyoxylate + acetyl-CoA + H2O = (S)-malate + CoA + H(+)
  3. transport of malate and sulfite (cytosol to mitochondria)

  Catalysed by AADAT, CLYBL, SLC25A10mitochondrial matrix, cytosolOne of 2 routes

• CHEBI:17380Reach from here →
  1. Phenylalanine Transaminase, Mitochondrial
  2. transport of phenylalanine and tryptophan (cytosol to extracellular)
  3. Transport of 3-Hydroxy-L-Kynurenine

  Catalysed by GOT2, TAT, SLC7A5mitochondrial matrix, extracellular space, cytosolOne of 5 routes

• (S)-1-pyrroline-5-carboxylateReach from here →
  1. Phenylalanine Transaminase, Mitochondrial
  2. transport of phenylalanine and proline (cytosol to extracellular)
  3. L-proline:(acceptor) oxidoreductase

  Catalysed by GOT2, TAT, SLC7A5, PRODH2mitochondrial matrix, extracellular space, cytosolOne of 2 routes

• agmatineReach from here →
  1. Phenylalanine Transaminase, Mitochondrial
  2. transport of arginine and phenylalanine (cytosol to extracellular)
  3. L-arginine carboxy-lyase (agmatine-forming)

  Catalysed by GOT2, TAT, SLC7A6mitochondrial matrix, extracellular space, cytosol

• enol-oxaloacetateReach from here →
  1. glyoxylate + L-phenylalanine = 3-phenylpyruvate + glycine
  2. glyoxylate + acetyl-CoA + H2O = (S)-malate + CoA + H(+)
  3. (S)-malate + a quinone = enol-oxaloacetate + a quinol

  Catalysed by AADAT, CLYBL, SDHB, SDHA

• AH2Reach from here →
  1. Phenylalanine Transaminase, Mitochondrial
  2. transport of alanine and phenylalanine (cytosol to extracellular)
  3. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O

  Catalysed by GOT2, TAT, SLC7A5, MOCOSmitochondrial matrix, extracellular space, cytosolOne of 4 routes

• 5-aminolevulinic acidReach from here →
  1. Phenylalanine Transaminase, Mitochondrial
  2. transport of glycine and phenylalanine (cytosol to extracellular)
  3. succinyl-CoA:glycine C-succinyltransferase (decarboxylating)

  Catalysed by GOT2, TAT, SLC7A5, ALAS1 and 2 moremitochondrial matrix, extracellular space, cytosol

• Ala-tRNA(Ala)Reach from here →
  1. Phenylalanine Transaminase, Mitochondrial
  2. transport of alanine and phenylalanine (cytosol to extracellular)
  3. L-Alanine:tRNA(Ala) ligase (AMP-forming)

  Catalysed by GOT2, TAT, SLC7A5, AARS1mitochondrial matrix, extracellular space, cytosol

• glycine betaineReach from here →
  1. Phenylalanine Transaminase, Mitochondrial
  2. Transport of L-Phenylalanine by Atb0 Transporter
  3. transport of betaine and chloride (cytosol to extracellular)

  Catalysed by GOT2, TAT, SLC6A14, SLC6A12mitochondrial matrix, extracellular space, cytosolOne of 3 routes

• trans-urocanateReach from here →
  1. Phenylalanine Transaminase, Mitochondrial
  2. transport of histidine and phenylalanine (cytosol to extracellular)
  3. L-histidine ammonia-lyase (urocanate-forming)

  Catalysed by GOT2, TAT, SLC7A6, HALmitochondrial matrix, extracellular space, cytosolOne of 2 routes

• urateReach from here →
  1. 3-phenylpyruvate + L-kynurenine = kynurenate + L-phenylalanine + H2O
  2. kynurenate(out) + glutarate(in) = kynurenate(in) + glutarate(out)
  3. urate(in) + glutarate(out) = urate(out) + glutarate(in)

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

• 5-hydroxy-L-tryptophanReach from here →
  1. Phenylalanine Transaminase, Mitochondrial
  2. transport of phenylalanine and tryptophan (cytosol to extracellular)
  3. (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

  Catalysed by GOT2, TAT, SLC7A5, TPH1 and 1 moremitochondrial matrix, extracellular space, cytosol

• CHEBI:17927Reach from here →
  1. Phenylalanine Transaminase, Mitochondrial
  2. transport of arginine and phenylalanine (cytosol to extracellular)
  3. Transport by Y+Lat-2 into Extracellular Space

  Catalysed by GOT2, TAT, SLC7A6, SLC3A2mitochondrial matrix, extracellular space, cytosol

• fumaric acidReach from here →
  1. glyoxylate + L-phenylalanine = 3-phenylpyruvate + glycine
  2. glyoxylate + acetyl-CoA + H2O = (S)-malate + CoA + H(+)
  3. (S)-malate hydro-lyase (fumarate-forming)

  Catalysed by AADAT, CLYBL, FHcytosolOne of 5 routes

• dTDPReach from here →
  1. glyoxylate + L-phenylalanine = 3-phenylpyruvate + glycine
  2. glyoxylate + L-alanine = glycine + pyruvate
  3. pyruvate kinase (dTDP)

  Catalysed by AADAT, AGXT, AGXT2, PKM and 1 morecytosol

• CHEBI:18095Reach from here →
  1. Phenylalanine Transaminase, Mitochondrial
  2. transport of alanine and phenylalanine (cytosol to extracellular)
  3. Transport of Hydroxy-Proline

  Catalysed by GOT2, TAT, SLC7A5, SLC1A4mitochondrial matrix, extracellular space, cytosolOne of 5 routes

• N-carbamoyl-beta-alanineReach from here →
  1. Phenylalanine Transaminase, Mitochondrial
  2. Transport of L-Phenylalanine by Atb0 Transporter
  3. Transport of 3-Ureidopropionate

  Catalysed by GOT2, TAT, SLC6A14mitochondrial matrix, extracellular space, cytosolOne of 2 routes

• N(1)-(5-phospho-D-ribosyl)glycinamideReach from here →
  1. Phenylalanine Transaminase, Mitochondrial
  2. transport of glycine and phenylalanine (cytosol to extracellular)
  3. 5-Phospho-D-ribosylamine:glycine ligase (ADP-forming)

  Catalysed by GOT2, TAT, SLC7A5, GARTmitochondrial matrix, extracellular space, cytosol

• 2-formamido-N(1)-(5-phospho-D-ribosyl)acetamidineReach from here →
  1. Phenylalanine Transaminase, Mitochondrial
  2. transport of glutamine and phenylalanine (cytosol to extracellular)
  3. 5'-Phosphoribosylformylglycinamide:L-glutamine amido-ligase (ADP-forming)

  Catalysed by GOT2, TAT, SLC7A5, PFASmitochondrial matrix, extracellular space, cytosol

• ophthalmateReach from here →
  1. Phenylalanine Transaminase, Mitochondrial
  2. transport of glycine and phenylalanine (cytosol to extracellular)
  3. gamma-L-glutamyl-(2S)-2-aminobutanoate + glycine + ATP = ophthalmate + ADP + phosphate + H(+)

  Catalysed by GOT2, TAT, SLC7A5, GSSmitochondrial matrix, extracellular space, cytosol

• 2-(alpha-Hydroxypropyl)thiamine diphosphateReach from here →
  1. glyoxylate + L-phenylalanine = 3-phenylpyruvate + glycine
  2. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  3. branched chain keto acid dehydrogenase E1 (2-oxobutyrate)

  Catalysed by AADAT, AGXT2, BCKDHA, BCKDHBmitochondrial matrix

• 5alpha-androstane-3alpha,17beta-diol 3-O-(beta-D-glucuronate)Reach from here →
  1. 3-phenylpyruvate + L-kynurenine = kynurenate + L-phenylalanine + H2O
  2. kynurenate(out) + glutarate(in) = kynurenate(in) + glutarate(out)
  3. 5alpha-androstane-3alpha,17beta-diol 3-O-(beta-D-glucuronate)(out) + glutarate(in) = 5alpha-androstane-3alpha,17beta-diol 3-O-(beta-D-glucuronate)(in) + glutarate(out)

  Catalysed by AADAT, SLC22A6, SLC22A24inner side of the membrane, outer side of the membraneOne of 2 routes

• 2-aminoisobutanoateReach from here →
  1. Phenylalanine Transaminase, Mitochondrial
  2. transport of alanine and phenylalanine (cytosol to extracellular)
  3. 2-aminoisobutanoate(out) + L-alanine(in) = 2-aminoisobutanoate(in) + L-alanine(out)

  Catalysed by GOT2, TAT, SLC7A5, SLC7A10mitochondrial matrix, extracellular space, cytosol, inner side of the membrane, outer side of the membraneOne of 2 routes

• 3-carboxy-4-methyl-5-propyl-2-furanpropanoateReach from here →
  1. 3-phenylpyruvate + L-kynurenine = kynurenate + L-phenylalanine + H2O
  2. kynurenate(out) + a dicarboxylate(in) = kynurenate(in) + a dicarboxylate(out)
  3. 3-carboxy-4-methyl-5-propyl-2-furanpropanoate(out) + a dicarboxylate(in) = 3-carboxy-4-methyl-5-propyl-2-furanpropanoate(in) + a dicarboxylate(out)

  Catalysed by AADAT, SLC22A8, SLC22A6inner side of the membrane, outer side of the membraneOne of 2 routes

• L-sepiapterinReach from here →
  1. 3-phenylpyruvate + L-kynurenine = kynurenate + L-phenylalanine + H2O
  2. kynurenate(out) + a dicarboxylate(in) = kynurenate(in) + a dicarboxylate(out)
  3. L-sepiapterin(out) + a dicarboxylate(in) = L-sepiapterin(in) + a dicarboxylate(out)

  Catalysed by AADAT, SLC22A8, SLC22A6inner side of the membrane, outer side of the membraneOne of 2 routes

• N(omega),N('omega)-dimethyl-L-arginineReach from here →
  1. Phenylalanine Transaminase, Mitochondrial
  2. transport of glycine and phenylalanine (cytosol to extracellular)
  3. N(omega),N('omega)-dimethyl-L-arginine + glyoxylate = 5-(3,3'-dimethylguanidino)-2-oxopentanoate + glycine

  Catalysed by GOT2, TAT, SLC7A5, AGXT2mitochondrial matrix, extracellular space, cytosolOne of 4 routes

• 5,6,7,8-tetrahydrofolic acidReach from here →
  1. Phenylalanine Transaminase, Mitochondrial
  2. transport of glycine and phenylalanine (cytosol to extracellular)
  3. 5,10-Methylenetetrahydrofolate:glycine hydroxymethyltransferase

  Catalysed by GOT2, TAT, SLC7A5, SHMT1mitochondrial matrix, extracellular space, cytosolOne of 2 routes

• S-disulfanyl-L-cysteineReach from here →
  1. Phenylalanine Transaminase, Mitochondrial
  2. transport of cysteine and phenylalanine (cytosol to extracellular)
  3. S-sulfanyl-L-cysteine + L-cysteine = S-disulfanyl-L-cysteine + L-alanine

  Catalysed by GOT2, TAT, SLC7A5, CARS2mitochondrial matrix, extracellular space, cytosol

• CHEBI:240943Reach from here →
  1. Phenylalanine Transaminase, Mitochondrial
  2. transport of glycine and phenylalanine (cytosol to extracellular)
  3. glycine N-acyltransferase (2-methylbutyryl-CoA)

  Catalysed by GOT2, TAT, SLC7A5, GLYAT and 2 moremitochondrial matrix, extracellular space, cytosolOne of 3 routes

• a quinolReach from here →
  1. glyoxylate + L-phenylalanine = 3-phenylpyruvate + glycine
  2. glyoxylate + acetyl-CoA + H2O = (S)-malate + CoA + H(+)
  3. (S)-malate + a quinone = enol-oxaloacetate + a quinol

  Catalysed by AADAT, CLYBL, SDHB, SDHA

• hypochlorous acidReach from here →
  1. Phenylalanine Transaminase, Mitochondrial
  2. Transport of L-Phenylalanine by Atb0 Transporter
  3. Peroxidase

  Catalysed by GOT2, TAT, SLC6A14, LPOmitochondrial matrix, extracellular space, cytosolOne of 4 routes

• potassium atomReach from here →
  1. Phenylalanine Transaminase, Mitochondrial
  2. Transport of L-Phenylalanine by Atb0 Transporter
  3. transport of chloride (cytosol to extracellular)

  Catalysed by GOT2, TAT, SLC6A14, SLC12A2 and 1 moremitochondrial matrix, extracellular space, cytosolOne of 3 routes

• phenylacetylglycineReach from here →
  1. Phenylalanine Transaminase, Mitochondrial
  2. transport of glycine and phenylalanine (cytosol to extracellular)
  3. phenylacetyl-CoA:glycine N-phenylacetyltransferase

  Catalysed by GOT2, TAT, SLC7A5, GLYATmitochondrial matrix, extracellular space, cytosolOne of 3 routes

• selenomethionineReach from here →
  1. glyoxylate + L-phenylalanine = 3-phenylpyruvate + glycine
  2. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  3. Selenomethionine methanethiol-lyase (deaminating)

  Catalysed by AADAT, AGXT2, CTHcytosol

• CHEBI:27760Reach from here →
  1. glyoxylate + L-phenylalanine = 3-phenylpyruvate + glycine
  2. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  3. Selenocystathionine Lysteine-lyase (deaminating)

  Catalysed by AADAT, AGXT2, CTHcytosol

• 5-hydroxytryptophanReach from here →
  1. Phenylalanine Transaminase, Mitochondrial
  2. transport of phenylalanine and tryptophan (cytosol to extracellular)
  3. L-Tryptophan, Tetrahydrobiopterin:Oxygen Oxidoreductase (5-Hydroxylating)

  Catalysed by GOT2, TAT, SLC7A5, TPH1 and 1 moremitochondrial matrix, extracellular space, cytosolOne of 2 routes

• 2-oxopentanoateReach from here →
  1. glyoxylate + L-phenylalanine = 3-phenylpyruvate + glycine
  2. N(omega),N(omega)-dimethyl-L-arginine + glyoxylate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + glycine
  3. 2-oxopentanoate + N(omega),N(omega)-dimethyl-L-arginine = 5-(3,3-dimethylguanidino)-2-oxopentanoate + L-2-aminopentanoate

  Catalysed by AADAT, AGXT2

• CHEBI:28666Reach from here →
  1. Phenylalanine Transaminase, Mitochondrial
  2. transport of glycine and phenylalanine (cytosol to extracellular)
  3. LTD4 hydrolysis

  Catalysed by GOT2, TAT, SLC7A5mitochondrial matrix, extracellular space, cytosol

• pyridoxine 5'-phosphateReach from here →
  1. Phenylalanine Transaminase, Mitochondrial
  2. transport of alanine and phenylalanine (cytosol to extracellular)
  3. L-selenocysteine selenide-lyase (L-alanine-forming)

  Catalysed by GOT2, TAT, SLC7A5, SCLYmitochondrial matrix, extracellular space, cytosol

• N-acetyl-L-cysteineReach from here →
  1. Phenylalanine Transaminase, Mitochondrial
  2. transport of cysteine and phenylalanine (cytosol to extracellular)
  3. N-acetylglutamate synthase (N-acetyl-L-cysteine)

  Catalysed by GOT2, TAT, SLC7A5, NAGSmitochondrial matrix, extracellular space, cytosol

• L-ascorbic acidReach from here →
  1. glyoxylate + L-phenylalanine = 3-phenylpyruvate + glycine
  2. N-octanoyl-(2S)-hydroxyglycine = octanamide + glyoxylate
  3. N-octanoylglycine + 2 L-ascorbate + O2 = N-octanoyl-(2S)-hydroxyglycine + 2 monodehydro-L-ascorbate radical + H2O

  Catalysed by AADAT, PAMOne of 6 routes

• K(+)Reach from here →
  1. Phenylalanine Transaminase, Mitochondrial
  2. transport of cysteine and phenylalanine (cytosol to extracellular)
  3. K(+)(in) + L-cysteine(out) + 3 Na(+)(out) + H(+)(out) = K(+)(out) + L-cysteine(in) + 3 Na(+)(in) + H(+)(in)

  Catalysed by GOT2, TAT, SLC7A5, SLC1A1mitochondrial matrix, extracellular space, cytosol, inner side of the membrane, outer side of the membraneOne of 4 routes

• 3-methyl-1-hydroxybutylthiamine diphosphateReach from here →
  1. glyoxylate + L-phenylalanine = 3-phenylpyruvate + glycine
  2. L-leucine + glyoxylate = 4-methyl-2-oxopentanoate + glycine
  3. branched chain keto acid dehydrogenase E1 (4-methyl-2-oxopentanoate)

  Catalysed by AADAT, BCKDHA, BCKDHBmitochondrial matrix

• Cys-tRNA(Cys)Reach from here →
  1. Phenylalanine Transaminase, Mitochondrial
  2. transport of cysteine and phenylalanine (cytosol to extracellular)
  3. L-Cysteine:tRNA(Cys) ligase (AMP-forming)

  Catalysed by GOT2, TAT, SLC7A5, CARS1mitochondrial matrix, extracellular space, cytosol

• Gly-tRNA(Gly)Reach from here →
  1. Phenylalanine Transaminase, Mitochondrial
  2. transport of glycine and phenylalanine (cytosol to extracellular)
  3. glycine:tRNA(Gly) ligase (AMP-forming)

  Catalysed by GOT2, TAT, SLC7A5, GARS1mitochondrial matrix, extracellular space, cytosol

• Asp-tRNA(Asp)Reach from here →
  1. Phenylalanine Transaminase, Mitochondrial
  2. L-aspartate(in) + L-phenylalanine(out) = L-aspartate(out) + L-phenylalanine(in)
  3. L-Aspartate:tRNA(Asp) ligase (AMP-forming)

  Catalysed by GOT2, TAT, SLC7A13, DARS1mitochondrial matrix, inner side of the membrane, outer side of the membrane, cytosol

• Trp-tRNA(Trp)Reach from here →
  1. Phenylalanine Transaminase, Mitochondrial
  2. transport of phenylalanine and tryptophan (cytosol to extracellular)
  3. L-Tryptophan -tRNA(Trp) ligase (AMP-forming)

  Catalysed by GOT2, TAT, SLC7A5, WARS1mitochondrial matrix, extracellular space, cytosol

• Ile-tRNA(Ile)Reach from here →
  1. Phenylalanine Transaminase, Mitochondrial
  2. transport of isoleucine and phenylalanine (cytosol to extracellular)
  3. L-Isoleucine:tRNA(Ile) ligase (AMP-forming)

  Catalysed by GOT2, TAT, SLC7A5, IARS1mitochondrial matrix, extracellular space, cytosol

• Gln-tRNA(Gln)Reach from here →
  1. Phenylalanine Transaminase, Mitochondrial
  2. transport of glutamine and phenylalanine (cytosol to extracellular)
  3. L-Glutamine:tRNA(Gln) ligase (AMP-forming)

  Catalysed by GOT2, TAT, SLC7A5, QARS1mitochondrial matrix, extracellular space, cytosol