4-methyl-2-oxopentanoate

Three steps

• 2-oxoglutaramateReach from here →
  1. 4-methyl-2-oxopentanoate + L-kynurenine = kynurenate + L-leucine + H2O
  2. transport of glutamine and leucine (cytosol to extracellular)
  3. L-Glutamine:pyruvate aminotransferase

  Catalysed by AADAT, SLC7A5, KYAT1extracellular space, cytosolOne of 6 routes

• enol-phenylpyruvateReach from here →
  1. L-leucine + glyoxylate = 4-methyl-2-oxopentanoate + glycine
  2. glyoxylate + L-phenylalanine = 3-phenylpyruvate + glycine
  3. 3-phenylpyruvate = enol-phenylpyruvate

  Catalysed by AADAT, MIF

• 3-fumarylpyruvateReach from here →
  1. L-leucine + glyoxylate = 4-methyl-2-oxopentanoate + glycine
  2. glyoxylate + L-alanine = glycine + pyruvate
  3. 3-fumarylpyruvate + H2O = fumarate + pyruvate + H(+)

  Catalysed by AADAT, AGXT, AGXT2, FAHD1

• CHEBI:16856Reach from here →
  1. 4-methyl-2-oxopentanoate + L-kynurenine = kynurenate + L-leucine + H2O
  2. transport of glycine and leucine (cytosol to extracellular)
  3. gamma-L-glutamyl-L-cysteine:glycine ligase (ADP-forming)

  Catalysed by AADAT, SLC7A5, GSSextracellular space, cytosolOne of 2 routes

• glyoxylic acidReach from here →
  1. 4-methyl-2-oxopentanoate + L-kynurenine = kynurenate + L-leucine + H2O
  2. transport of glycine and leucine (cytosol to extracellular)
  3. glycine:oxygen oxidoreductase (deaminating)

  Catalysed by AADAT, SLC7A5, DAOextracellular space, cytosol, peroxisomeOne of 4 routes

• N-acetyl-L-aspartateReach from here →
  1. 4-methyl-2-oxopentanoate + L-kynurenine = kynurenate + L-leucine + H2O
  2. L-leucine(out) + L-aspartate(in) = L-leucine(in) + L-aspartate(out)
  3. acetyl-CoA:L-aspartate N-acetyltransferase

  Catalysed by AADAT, SLC7A13, NAT8Linner side of the membrane, outer side of the membrane, mitochondrial matrixOne of 2 routes

• oxalic acidReach from here →
  1. 4-methyl-2-oxopentanoate + L-kynurenine = kynurenate + L-leucine + H2O
  2. transport of chloride and leucine (cytosol to extracellular)
  3. transport of chloride and oxalate (cytosol to extracellular)

  Catalysed by AADAT, SLC6A14, SLC26A6extracellular space, cytosol

• (R)-3-hydroxybutyric acidReach from here →
  1. L-leucine + glyoxylate = 4-methyl-2-oxopentanoate + glycine
  2. glyoxylate + L-alanine = glycine + pyruvate
  3. transport of (R)-3-hydroxybutanoate and pyruvate (cytosol to extracellular)

  Catalysed by AADAT, AGXT, AGXT2, SLC16A4 and 5 moreextracellular space, cytosolOne of 2 routes

• 3-hydroxypyruvateReach from here →
  1. L-leucine + glyoxylate = 4-methyl-2-oxopentanoate + glycine
  2. glyoxylate + L-alanine = glycine + pyruvate
  3. L-serine + pyruvate = 3-hydroxypyruvate + L-alanine

  Catalysed by AADAT, AGXT, AGXT2

• (S)-4-hydroxymandelateReach from here →
  1. L-leucine + glyoxylate = 4-methyl-2-oxopentanoate + glycine
  2. L-tyrosine + glyoxylate = 3-(4-hydroxyphenyl)pyruvate + glycine
  3. 3-(4-hydroxyphenyl)pyruvate + O2 = (S)-4-hydroxymandelate + CO2

  Catalysed by AADAT, HPDL

• L-glutamic 5-semialdehydeReach from here →
  1. 4-methyl-2-oxopentanoate + L-kynurenine = kynurenate + L-leucine + H2O
  2. transport of leucine and ornithine (cytosol to extracellular)
  3. L-Ornithine:2-oxo-acid aminotransferase

  Catalysed by AADAT, SLC7A6, OATextracellular space, cytosol, mitochondrial matrix

• itaconateReach from here →
  1. 4-methyl-2-oxopentanoate + L-kynurenine = kynurenate + L-leucine + H2O
  2. kynurenate(out) + glutarate(in) = kynurenate(in) + glutarate(out)
  3. itaconate + glutaryl-CoA = itaconyl-CoA + glutarate

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

• anilineReach from here →
  1. 4-methyl-2-oxopentanoate + L-kynurenine = kynurenate + L-leucine + H2O
  2. Acetylation of Leucine
  3. Acetyl-CoA:arylamine N-acetyltransferase

  Catalysed by AADAT, NAT2, NAT1mitochondrial matrix, cytosol

• aceneuramateReach from here →
  1. L-leucine + glyoxylate = 4-methyl-2-oxopentanoate + glycine
  2. glyoxylate + L-alanine = glycine + pyruvate
  3. aceneuramate = aldehydo-N-acetyl-D-mannosamine + pyruvate

  Catalysed by AADAT, AGXT, AGXT2, NPL

• CHEBI:17312Reach from here →
  1. 4-methyl-2-oxopentanoate + L-kynurenine = kynurenate + L-leucine + H2O
  2. Acetylation of Leucine
  3. N1,N12-diacetylspermine to N1-acetylspermine conversion

  Catalysed by AADATmitochondrial matrix, cytosolOne of 2 routes

• 5'-deoxyadenosineReach from here →
  1. L-leucine + glyoxylate = 4-methyl-2-oxopentanoate + glycine
  2. glyoxylate + L-alanine = glycine + pyruvate
  3. N(1)-methylguanosine(37) in tRNA(Phe) + pyruvate + S-adenosyl-L-methionine = 4-demethylwyosine(37) in tRNA(Phe) + 5'-deoxyadenosine + L-methionine + CO2 + H2O

  Catalysed by AADAT, AGXT, AGXT2, TYW1B and 1 more

• 3-hydroxy-3-methylglutarateReach from here →
  1. 4-methyl-2-oxopentanoate + L-kynurenine = kynurenate + L-leucine + 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. L-leucine + glyoxylate = 4-methyl-2-oxopentanoate + 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. 4-methyl-2-oxopentanoate + L-kynurenine = kynurenate + L-leucine + H2O
  2. transport of leucine and tryptophan (cytosol to extracellular)
  3. Transport of 3-Hydroxy-L-Kynurenine

  Catalysed by AADAT, SLC7A5extracellular space, cytosolOne of 5 routes

• (S)-1-pyrroline-5-carboxylateReach from here →
  1. 4-methyl-2-oxopentanoate + L-kynurenine = kynurenate + L-leucine + H2O
  2. transport of leucine and proline (cytosol to extracellular)
  3. L-proline:(acceptor) oxidoreductase

  Catalysed by AADAT, SLC7A5, PRODH2extracellular space, cytosolOne of 2 routes

• agmatineReach from here →
  1. 4-methyl-2-oxopentanoate + L-kynurenine = kynurenate + L-leucine + H2O
  2. transport of arginine and leucine (cytosol to extracellular)
  3. L-arginine carboxy-lyase (agmatine-forming)

  Catalysed by AADAT, SLC3A2, SLC7A6, SLC7A7extracellular space, cytosol, mitochondrial matrix

• enol-oxaloacetateReach from here →
  1. L-leucine + glyoxylate = 4-methyl-2-oxopentanoate + 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. 4-methyl-2-oxopentanoate + L-kynurenine = kynurenate + L-leucine + H2O
  2. transport of alanine and leucine (cytosol to extracellular)
  3. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O

  Catalysed by AADAT, SLC7A5, MOCOSextracellular space, cytosolOne of 4 routes

• 5-aminolevulinic acidReach from here →
  1. 4-methyl-2-oxopentanoate + L-kynurenine = kynurenate + L-leucine + H2O
  2. transport of glycine and leucine (cytosol to extracellular)
  3. succinyl-CoA:glycine C-succinyltransferase (decarboxylating)

  Catalysed by AADAT, SLC7A5, ALAS1, ALAS2 and 1 moreextracellular space, cytosol, mitochondrial matrix

• (7Z,10Z,13Z)-hexadecatrienoyl-CoAReach from here →
  1. 4-methyl-2-oxopentanoate + L-kynurenine = kynurenate + L-leucine + H2O
  2. Acetylation of Leucine
  3. Fatty Acid Beta Oxidation (C18:3->C16:3), Mitochondrial

  Catalysed by AADAT, HADHA, ACADVL, HADHBmitochondrial matrix

• Ala-tRNA(Ala)Reach from here →
  1. 4-methyl-2-oxopentanoate + L-kynurenine = kynurenate + L-leucine + H2O
  2. transport of alanine and leucine (cytosol to extracellular)
  3. L-Alanine:tRNA(Ala) ligase (AMP-forming)

  Catalysed by AADAT, SLC7A5, AARS1extracellular space, cytosol

• glycine betaineReach from here →
  1. 4-methyl-2-oxopentanoate + L-kynurenine = kynurenate + L-leucine + H2O
  2. transport of chloride and leucine (cytosol to extracellular)
  3. transport of betaine and chloride (cytosol to extracellular)

  Catalysed by AADAT, SLC6A14, SLC6A12extracellular space, cytosolOne of 3 routes

• trans-urocanateReach from here →
  1. 4-methyl-2-oxopentanoate + L-kynurenine = kynurenate + L-leucine + H2O
  2. transport of histidine and leucine (cytosol to extracellular)
  3. L-histidine ammonia-lyase (urocanate-forming)

  Catalysed by AADAT, SLC7A6, HALextracellular space, cytosol

• urateReach from here →
  1. 4-methyl-2-oxopentanoate + L-kynurenine = kynurenate + L-leucine + 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. 4-methyl-2-oxopentanoate + L-kynurenine = kynurenate + L-leucine + H2O
  2. transport of leucine 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 AADAT, SLC7A5, TPH1, TPH2extracellular space, cytosol

• CHEBI:17927Reach from here →
  1. 4-methyl-2-oxopentanoate + L-kynurenine = kynurenate + L-leucine + H2O
  2. transport of arginine and leucine (cytosol to extracellular)
  3. Transport by Y+Lat-2 into Extracellular Space

  Catalysed by AADAT, SLC3A2, SLC7A6, SLC7A7extracellular space, cytosolOne of 2 routes

• fumaric acidReach from here →
  1. L-leucine + glyoxylate = 4-methyl-2-oxopentanoate + 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. L-leucine + glyoxylate = 4-methyl-2-oxopentanoate + 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. 4-methyl-2-oxopentanoate + L-kynurenine = kynurenate + L-leucine + H2O
  2. transport of alanine and leucine (cytosol to extracellular)
  3. Transport of Hydroxy-Proline

  Catalysed by AADAT, SLC7A5, SLC1A4extracellular space, cytosolOne of 5 routes

• N-carbamoyl-beta-alanineReach from here →
  1. 4-methyl-2-oxopentanoate + L-kynurenine = kynurenate + L-leucine + H2O
  2. transport of chloride and leucine (cytosol to extracellular)
  3. Transport of 3-Ureidopropionate

  Catalysed by AADAT, SLC6A14extracellular space, cytosolOne of 2 routes

• N(1)-(5-phospho-D-ribosyl)glycinamideReach from here →
  1. 4-methyl-2-oxopentanoate + L-kynurenine = kynurenate + L-leucine + H2O
  2. transport of glycine and leucine (cytosol to extracellular)
  3. 5-Phospho-D-ribosylamine:glycine ligase (ADP-forming)

  Catalysed by AADAT, SLC7A5, GARTextracellular space, cytosol

• 2-phenylethylamineReach from here →
  1. 4-methyl-2-oxopentanoate + L-kynurenine = kynurenate + L-leucine + H2O
  2. transport of leucine and phenylalanine (cytosol to extracellular)
  3. L-phenylalanine carboxy-lyase (phenylethylamine-forming)

  Catalysed by AADAT, SLC7A5, DDCextracellular space, cytosol

• 2-formamido-N(1)-(5-phospho-D-ribosyl)acetamidineReach from here →
  1. 4-methyl-2-oxopentanoate + L-kynurenine = kynurenate + L-leucine + H2O
  2. transport of glutamine and leucine (cytosol to extracellular)
  3. 5'-Phosphoribosylformylglycinamide:L-glutamine amido-ligase (ADP-forming)

  Catalysed by AADAT, SLC7A5, PFASextracellular space, cytosol

• ophthalmateReach from here →
  1. 4-methyl-2-oxopentanoate + L-kynurenine = kynurenate + L-leucine + H2O
  2. transport of glycine and leucine (cytosol to extracellular)
  3. gamma-L-glutamyl-(2S)-2-aminobutanoate + glycine + ATP = ophthalmate + ADP + phosphate + H(+)

  Catalysed by AADAT, SLC7A5, GSSextracellular space, cytosol

• 2-(alpha-Hydroxypropyl)thiamine diphosphateReach from here →
  1. branched chain keto acid dehydrogenase E1 (4-methyl-2-oxopentanoate)
  2. lipoamide to S-(3-methylbutanoyl)... conversion
  3. branched chain keto acid dehydrogenase E1 (2-oxobutyrate)

  Catalysed by BCKDHA, BCKDHB, DBTmitochondrial matrixOne of 2 routes

• 5alpha-androstane-3alpha,17beta-diol 3-O-(beta-D-glucuronate)Reach from here →
  1. 4-methyl-2-oxopentanoate + L-kynurenine = kynurenate + L-leucine + 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. 4-methyl-2-oxopentanoate + L-kynurenine = kynurenate + L-leucine + H2O
  2. transport of alanine and leucine (cytosol to extracellular)
  3. 2-aminoisobutanoate(out) + L-alanine(in) = 2-aminoisobutanoate(in) + L-alanine(out)

  Catalysed by AADAT, SLC7A5, SLC7A10extracellular 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. 4-methyl-2-oxopentanoate + L-kynurenine = kynurenate + L-leucine + 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. 4-methyl-2-oxopentanoate + L-kynurenine = kynurenate + L-leucine + 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

• biocytinReach from here →
  1. 4-methyl-2-oxopentanoate + L-kynurenine = kynurenate + L-leucine + H2O
  2. L-lysine(in) + L-leucine(out) + Na(+)(out) = L-lysine(out) + L-leucine(in) + Na(+)(in)
  3. biocytin + H2O = biotin + L-lysine

  Catalysed by AADAT, SLC7A7, BTDinner side of the membrane, outer side of the membrane

• N(omega),N('omega)-dimethyl-L-arginineReach from here →
  1. 4-methyl-2-oxopentanoate + L-kynurenine = kynurenate + L-leucine + H2O
  2. transport of glycine and leucine (cytosol to extracellular)
  3. N(omega),N('omega)-dimethyl-L-arginine + glyoxylate = 5-(3,3'-dimethylguanidino)-2-oxopentanoate + glycine

  Catalysed by AADAT, SLC7A5, AGXT2extracellular space, cytosolOne of 4 routes

• 5,6,7,8-tetrahydrofolic acidReach from here →
  1. 4-methyl-2-oxopentanoate + L-kynurenine = kynurenate + L-leucine + H2O
  2. transport of glycine and leucine (cytosol to extracellular)
  3. 5,10-Methylenetetrahydrofolate:glycine hydroxymethyltransferase

  Catalysed by AADAT, SLC7A5, SHMT1extracellular space, cytosolOne of 2 routes

• S-disulfanyl-L-cysteineReach from here →
  1. 4-methyl-2-oxopentanoate + L-kynurenine = kynurenate + L-leucine + H2O
  2. transport of cysteine and leucine (cytosol to extracellular)
  3. S-sulfanyl-L-cysteine + L-cysteine = S-disulfanyl-L-cysteine + L-alanine

  Catalysed by AADAT, SLC7A9, SLC3A1, CARS2extracellular space, cytosol

• (5Z)-dodecenoyl-CoAReach from here →
  1. 4-methyl-2-oxopentanoate + L-kynurenine = kynurenate + L-leucine + H2O
  2. Acetylation of Leucine
  3. acetyl-CoA C-acyltransferase (3-oxomyrist-7-enoyl-CoA)

  Catalysed by AADAT, HADHA, ACAA2, HADHBmitochondrial matrixOne of 2 routes

• (11Z)-docosenoyl-CoA(4-)Reach from here →
  1. 4-methyl-2-oxopentanoate + L-kynurenine = kynurenate + L-leucine + H2O
  2. Acetylation of Leucine
  3. long-chain-fatty-acid-CoA ligase (cis-cetoleic acid)

  Catalysed by AADAT, SLC27A2, ACSL4, ACSL3 and 5 moremitochondrial matrix, cytosolOne of 5 routes