2-oxobutanoate

Three steps

• L-aspartateReach from here →
  1. L-threonine = 2-oxobutanoate + NH4(+)
  2. L-threonine(in) + L-glutamine(out) + Na(+)(out) = L-threonine(out) + L-glutamine(in) + Na(+)(in)
  3. L-aspartate(in) + L-glutamine(out) = L-aspartate(out) + L-glutamine(in)

  Catalysed by SDS, SDSL, SLC1A5, SLC7A13inner side of the membrane, outer side of the membraneOne of 11 routes

• citric acidReach from here →
  1. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  2. glyoxylate + acetyl-CoA + H2O = (S)-malate + CoA + H(+)
  3. Citrate Transport, Mitochondrial

  Catalysed by AGXT2, CLYBL, SLC25A1mitochondrial matrix, cytosolOne of 9 routes

• (9Z)-octadecenoateReach from here →
  1. 2-oxobutyrate oxidation
  2. Active Transport
  3. Utilized Transport

  No catalyst recordedcytosol, mitochondrial matrix, endoplasmic reticulumOne of 2 routes

• adipic acidReach from here →
  1. 2-oxobutanoate + L-kynurenine = (2S)-2-aminobutanoate + kynurenate + H2O
  2. kynurenate(out) + glutarate(in) = kynurenate(in) + glutarate(out)
  3. hexanedioate + glutaryl-CoA = hexanedioyl-CoA + glutarate

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

• (indol-3-yl)acetateReach from here →
  1. 2-oxobutanoate + L-kynurenine = (2S)-2-aminobutanoate + kynurenate + H2O
  2. kynurenate(out) + a dicarboxylate(in) = kynurenate(in) + a dicarboxylate(out)
  3. (indol-3-yl)acetate(out) + a dicarboxylate(in) = (indol-3-yl)acetate(in) + a dicarboxylate(out)

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

• isocitric acidReach from here →
  1. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  2. glyoxylate + acetyl-CoA + H2O = (S)-malate + CoA + H(+)
  3. Mitochondrial Carrier (Mc) Tcdb:2.A.29.7.2

  Catalysed by AGXT2, CLYBL, SLC25A1mitochondrial matrix, cytosolOne of 5 routes

• 4-hydroxy-2-oxoglutaric acidReach from here →
  1. N(omega),N(omega)-dimethyl-L-arginine + 2-oxobutanoate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + (2S)-2-aminobutanoate
  2. N(omega),N(omega)-dimethyl-L-arginine + oxaloacetate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + L-aspartate
  3. D-aspartate:2-oxoglutarate aminotransferase

  Catalysed by AGXT2, GOT2mitochondrial matrix

• (9Z)-hexadecenoateReach from here →
  1. 2-oxobutyrate oxidation
  2. Active Transport
  3. Utilized Transport

  No catalyst recordedcytosol, mitochondrial matrix, endoplasmic reticulumOne of 6 routes

• (5Z,8Z,11Z,14Z)-eicosatetraenoateReach from here →
  1. 2-oxobutyrate oxidation
  2. Active Transport
  3. Utilized Transport

  No catalyst recordedcytosol, mitochondrial matrix, endoplasmic reticulumOne of 2 routes

• L-lysineReach from here →
  1. 2-oxobutanoate + L-kynurenine = (2S)-2-aminobutanoate + kynurenate + H2O
  2. N(omega),N(omega)-dimethyl-L-arginine + 2-oxobutanoate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + (2S)-2-aminobutanoate
  3. N(omega),N(omega)-dimethyl-L-arginine(in) + L-lysine(out) = N(omega),N(omega)-dimethyl-L-arginine(out) + L-lysine(in)

  Catalysed by AADAT, AGXT2, SLC25A2inner side of the membrane, outer side of the membraneOne of 2 routes

• cis-aconitic acidReach from here →
  1. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  2. glyoxylate + acetyl-CoA + H2O = (S)-malate + CoA + H(+)
  3. Mitochondrial Carrier (Mc) Tcdb:2.A.29.7.2

  Catalysed by AGXT2, CLYBL, SLC25A1mitochondrial matrix, cytosolOne of 5 routes

• thiosulfateReach from here →
  1. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  2. glyoxylate + acetyl-CoA + H2O = (S)-malate + CoA + H(+)
  3. transport of malate and thiosulfate (cytosol to mitochondria)

  Catalysed by AGXT2, CLYBL, SLC25A10mitochondrial matrix, cytosolOne of 4 routes

• 4-methylthio-2-oxobutanoic acidReach from here →
  1. 2-oxobutanoate + L-alanine = (2S)-2-aminobutanoate + pyruvate
  2. N(1)-methylguanosine(37) in tRNA(Phe) + pyruvate + S-adenosyl-L-methionine = 4-demethylwyosine(37) in tRNA(Phe) + 5'-deoxyadenosine + L-methionine + CO2 + H2O
  3. L-Methionine:oxygen oxidoreductase (deaminating)

  Catalysed by AGXT2, TYW1B, TYW1, IL4I1cytosol

• dodecanamideReach from here →
  1. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  2. N-dodecanoyl-(2S)-hydroxyglycine = dodecanamide + glyoxylate
  3. N-dodecanoyl-(2S)-hydroxyglycine = dodecanamide + glyoxylate

  Catalysed by AGXT2, PAM

• D-cysteineReach from here →
  1. L-threonine = 2-oxobutanoate + NH4(+)
  2. L-threonine(out) + L-alanine(in) = L-threonine(in) + L-alanine(out)
  3. D-cysteine(out) + L-alanine(in) = D-cysteine(in) + L-alanine(out)

  Catalysed by SDS, SDSL, SLC7A10inner side of the membrane, outer side of the membraneOne of 2 routes

• 5-aminolevulinateReach from here →
  1. L-threonine = 2-oxobutanoate + NH4(+)
  2. L-threonine = acetaldehyde + glycine
  3. succinyl-CoA + glycine + H(+) = 5-aminolevulinate + CO2 + CoA

  Catalysed by SDS, SDSL, SHMT1, ALAS1 and 1 more

• glycochenodeoxycholateReach from here →
  1. L-threonine = 2-oxobutanoate + NH4(+)
  2. L-threonine = acetaldehyde + glycine
  3. chenodeoxycholoyl-CoA + glycine = glycochenodeoxycholate + CoA + H(+)

  Catalysed by SDS, SDSL, SHMT1, BAAT

• taurocholateReach from here →
  1. 2-oxobutanoate + L-kynurenine = (2S)-2-aminobutanoate + kynurenate + H2O
  2. kynurenate(out) + glutarate(in) = kynurenate(in) + glutarate(out)
  3. taurocholate(out) + glutarate(in) = taurocholate(in) + glutarate(out)

  Catalysed by AADAT, SLC22A6, SLC22A24, SLC22A8inner side of the membrane, outer side of the membraneOne of 2 routes

• glycochenodeoxycholic acidReach from here →
  1. L-threonine = 2-oxobutanoate + NH4(+)
  2. L-threonine = acetaldehyde + glycine
  3. bile acid-CoA: amino acid N-acyltransferase

  Catalysed by SDS, SDSL, SHMT1, BAATperoxisomeOne of 3 routes

• 3-hydroxyisovaleric acidReach from here →
  1. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  2. L-leucine + glyoxylate = 4-methyl-2-oxopentanoate + glycine
  3. 4-methyl-2-oxopentanoate decarboxylation

  Catalysed by AGXT2, AADAT, ALOX12B, ALOXE3cytosol

• glycolithocholic acidReach from here →
  1. L-threonine = 2-oxobutanoate + NH4(+)
  2. L-threonine = acetaldehyde + glycine
  3. bile acid-CoA:amino acid N-acyltransferase (glycine)

  Catalysed by SDS, SDSL, SHMT1, BAATcytosolOne of 2 routes

• decanamideReach from here →
  1. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  2. N-decanoyl-(2S)-hydroxyglycine = decanamide + glyoxylate
  3. N-decanoyl-(2S)-hydroxyglycine = decanamide + glyoxylate

  Catalysed by AGXT2, PAM

• L-2-amino-3-oxobutanoic acidReach from here →
  1. L-threonine = 2-oxobutanoate + NH4(+)
  2. L-threonine = acetaldehyde + glycine
  3. Acetyl-CoA:glycine C-acetyltransferase

  Catalysed by SDS, SDSL, SHMT1, GCATmitochondrial matrix

• L-erythro-7,8-dihydrobiopterinReach from here →
  1. 2-oxobutanoate + L-kynurenine = (2S)-2-aminobutanoate + kynurenate + H2O
  2. kynurenate(out) + a dicarboxylate(in) = kynurenate(in) + a dicarboxylate(out)
  3. L-erythro-7,8-dihydrobiopterin(out) + a dicarboxylate(in) = L-erythro-7,8-dihydrobiopterin(in) + a dicarboxylate(out)

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

• (3S)-3-hydroxy-3-methylglutaryl-CoAReach from here →
  1. 2-hydroxybutyrate:NAD+ oxidoreductase
  2. transport of 2-hydroxybutyrate and acetoacetate (cytosol to extracellular)
  3. (3S)-3-hydroxy-3-methylglutaryl-CoA = acetoacetate + acetyl-CoA

  Catalysed by LDHB, LDHC, LDHAL6A, LDHAL6B and 6 morecytosol, extracellular space

• 13-cis-retinalReach from here →
  1. 2-hydroxybutyrate:NAD+ oxidoreductase
  2. transport of 2-hydroxybutyrate and retinoate (cytosol to extracellular)
  3. retinal dehydrogenase (13-cis-retinal)

  Catalysed by LDHB, LDHC, LDHAL6A, LDHAL6B and 6 morecytosol, extracellular space, endoplasmic reticulum

• L-ornithineReach from here →
  1. L-threonine = 2-oxobutanoate + NH4(+)
  2. L-threonine = acetaldehyde + glycine
  3. L-Arginine:glycine amidinotransferase

  Catalysed by SDS, SDSL, SHMT1, GATMcytosolOne of 16 routes

• N-(acetyl)-sphing-4-enineReach from here →
  1. 2-hydroxybutyrate:NAD+ oxidoreductase
  2. transport of 2-hydroxybutyrate and acetate (cytosol to extracellular)
  3. N-(acetyl)-sphing-4-enine + H2O = sphing-4-enine + acetate

  Catalysed by LDHB, LDHC, LDHAL6A, LDHAL6B and 5 morecytosol, extracellular space

• 2-methyl-1-hydroxypropylthiamine diphosphateReach from here →
  1. branched chain keto acid dehydrogenase E1 (2-oxobutyrate)
  2. lipoamide to thiamin-PP conversion
  3. branched chain keto acid dehydrogenase E1 (3-methyl-2-oxobutyrate)

  Catalysed by BCKDHA, BCKDHB, DBTmitochondrial matrix

• 1,2-dihydroxy-5-(methylsulfanyl)pent-1-en-3-oneReach from here →
  1. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  2. glyoxylate + L-methionine = 4-methylsulfanyl-2-oxobutanoate + glycine
  3. 1,2-dihydroxy-5-(methylsulfanyl)pent-1-en-3-one + O2 = 4-methylsulfanyl-2-oxobutanoate + formate + 2 H(+)

  Catalysed by AGXT2, AADAT, ADI1

• gamma-Glu-AlaReach from here →
  1. L-threonine = 2-oxobutanoate + NH4(+)
  2. L-threonine(out) + L-alanine(in) = L-threonine(in) + L-alanine(out)
  3. gamma-glutamyltransferase (alanine)

  Catalysed by SDS, SDSL, SLC7A10, GGT1 and 5 moreinner side of the membrane, outer side of the membrane, extracellular space, cytosol

• S(8)-aminomethyldihydrolipoamideReach from here →
  1. L-threonine = 2-oxobutanoate + NH4(+)
  2. L-threonine = acetaldehyde + glycine
  3. dihydrolipoyl dehydrogenase ([protein]-N6-(lipoyl)lysine)

  Catalysed by SDS, SDSL, SHMT1, GLDC and 1 moremitochondrial matrix

• taurineReach from here →
  1. 2-oxobutyrate oxidation
  2. Propanoyl-CoA hydrolase
  3. bile acid-CoA:amino acid N-acyltransferase (taurodeoxycholate)

  Catalysed by ACOT7, BAATcytosolOne of 2 routes

• acetoacetyl-CoAReach from here →
  1. 2-hydroxybutyrate:NAD+ oxidoreductase
  2. transport of 2-hydroxybutyrate and acetoacetate (cytosol to extracellular)
  3. acetoacetate + ATP + CoA = acetoacetyl-CoA + AMP + diphosphate

  Catalysed by LDHB, LDHC, LDHAL6A, LDHAL6B and 5 morecytosol, extracellular spaceOne of 2 routes

• (S)-malyl-CoAReach from here →
  1. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  2. glyoxylate + acetyl-CoA + H2O = (S)-malate + CoA + H(+)
  3. (S)-malate + ATP + CoA = (S)-malyl-CoA + ADP + phosphate

  Catalysed by AGXT2, CLYBL, SUCLG1, SUCLA2

• (R)-methylmalonyl-CoAReach from here →
  1. 2-oxobutyrate oxidation
  2. Propanoyl-CoA hydrolase
  3. aldehyde dehydrogenase (2-methyl-3-oxopropanoate)

  Catalysed by ACOT7, ALDH6A1cytosol, mitochondrial matrixOne of 5 routes

• 3'-dephospho-CoAReach from here →
  1. 2-oxobutyrate oxidation
  2. Propanoyl-CoA hydrolase
  3. acid phosphatase 2, lysosomal (CoA)

  Catalysed by ACOT7, ACP2cytosol, lysosomeOne of 2 routes

• (2E)-dodecenoyl-CoAReach from here →
  1. 2-oxobutyrate oxidation
  2. Propanoyl-CoA hydrolase
  3. (2E)-dodecenoyl-CoA formation

  Catalysed by ACOT7cytosol, mitochondrial matrixOne of 3 routes

• 4,8,12-trimethyltridecanoyl-CoAReach from here →
  1. 2-oxobutyrate oxidation
  2. Propanoyl-CoA hydrolase
  3. propanoyl-CoA C-acyltransferase (3-ketopristanoyl-CoA)

  Catalysed by ACOT7, ACAA1, MYO5Bcytosol, peroxisomeOne of 2 routes

• (6Z,9Z,12Z)-octadecatrienoyl-CoAReach from here →
  1. 2-oxobutyrate oxidation
  2. Propanoyl-CoA hydrolase
  3. long-chain-fatty-acid-CoA ligase (gamma-linolenate)

  Catalysed by ACOT7, SLC27A2, ACSL4, ACSL3 and 5 morecytosolOne of 7 routes

• butanoyl-CoAReach from here →
  1. 2-hydroxybutyrate:NAD+ oxidoreductase
  2. transport of 2-hydroxybutyrate and butyrate (cytosol to extracellular)
  3. Butanoate:CoA ligase (AMP-forming)

  Catalysed by LDHB, LDHC, LDHAL6A, LDHAL6B and 10 morecytosol, extracellular spaceOne of 13 routes

• dodecanoyl-CoAReach from here →
  1. 2-oxobutyrate oxidation
  2. Propanoyl-CoA hydrolase
  3. long-chain-fatty-acid-CoA ligase (lauric acid)

  Catalysed by ACOT7, SLC27A2, ACSL4, ACSL3 and 5 morecytosolOne of 9 routes

• glutaryl-CoAReach from here →
  1. 2-oxobutyrate oxidation
  2. Propanoyl-CoA hydrolase
  3. Production of Glutaryl Carnitine

  Catalysed by ACOT7, CPT1A, CPT1C, CPT1BcytosolOne of 6 routes

• eicosanoyl-CoAReach from here →
  1. 2-oxobutyrate oxidation
  2. Propanoyl-CoA hydrolase
  3. long-chain-fatty-acid-CoA ligase (eicosanoate)

  Catalysed by ACOT7, SLC27A2, ACSL4, ACSL3 and 5 morecytosolOne of 12 routes

• itaconyl-CoAReach from here →
  1. 2-oxobutyrate oxidation
  2. Propanoyl-CoA hydrolase
  3. Itaconate:CoA ligase (GDP-forming)

  Catalysed by ACOT7, SUCLG1, SUCLG2, SUCLA2cytosol, mitochondrial matrixOne of 2 routes

• (9Z,12Z)-octadecadienoyl-CoAReach from here →
  1. 2-oxobutyrate oxidation
  2. Propanoyl-CoA hydrolase
  3. long-chain-fatty-acid-CoA ligase ((6Z,9Z)-octadecadienoic acid)

  Catalysed by ACOT7, SLC27A2, ACSL4, ACSL3 and 5 morecytosolOne of 13 routes

• tetradecanoyl-CoAReach from here →
  1. 2-oxobutyrate oxidation
  2. Propanoyl-CoA hydrolase
  3. long-chain-fatty-acid-CoA ligase (myristic acid)

  Catalysed by ACOT7, SLC27A2, ACSL4, ACSL3 and 5 morecytosolOne of 11 routes

• octanoyl-CoAReach from here →
  1. 2-oxobutyrate oxidation
  2. Propanoyl-CoA hydrolase
  3. Octanoyl-CoA:L-carnitine O-octanoyltransferase

  Catalysed by ACOT7, CROTcytosol, mitochondrial matrixOne of 20 routes

• phytanoyl-CoAReach from here →
  1. 2-oxobutyrate oxidation
  2. Propanoyl-CoA hydrolase
  3. phytanate-CoA ligase

  Catalysed by ACOT7cytosolOne of 5 routes

• propanoyl-CoAReach from here →
  1. 2-oxobutyrate oxidation
  2. propanoyl-CoA:carbon-dioxide ligase (ADP-forming)
  3. (S)-methylmalonyl-CoA + H(+) = propanoyl-CoA + CO2

  Catalysed by PCCA, PCCB, ECHDC1cytosol, mitochondrial matrixOne of 3 routes