L-2-amino-3-oxobutanoic acid

Three steps

• oxalateReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. glycine + 2-oxoglutarate = glyoxylate + L-glutamate
  3. glyoxylate + O2 + H2O = oxalate + H2O2 + H(+)

  Catalysed by GCAT, AADAT, HAO1mitochondrial matrix

• formic acidReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. transport of chloride and glycine (cytosol to extracellular)
  3. transport of chloride and formate (cytosol to extracellular)

  Catalysed by GCAT, SLC6A9, SLC6A14, SLC26A4mitochondrial matrix, extracellular space, cytosolOne of 2 routes

• malonic acidReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. transport of chloride and glycine (cytosol to extracellular)
  3. Malonate Transport, Antiport with Chloride

  Catalysed by GCAT, SLC6A9, SLC6A14mitochondrial matrix, extracellular space, cytosolOne of 2 routes

• (9Z)-octadecenoateReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. (9Z)-octadecenoyl-CoA + glycine = N-(9Z-octadecenoyl)glycine + CoA + H(+)
  3. (9Z)-octadecenoate + glycine = N-(9Z-octadecenoyl)glycine + H2O

  Catalysed by GCAT, GLYATL3, GLYATL2, FAAH and 1 moremitochondrial matrix

• an alcoholReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. gamma-L-glutamyl-L-cysteine + glycine + ATP = glutathione + ADP + phosphate + H(+)
  3. a hydroperoxide + 2 glutathione = an alcohol + glutathione disulfide + H2O

  Catalysed by GCAT, GSS, CP, PRDX6mitochondrial matrix

• glutarateReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. bile acid-CoA: amino acid N-acyltransferase
  3. glycocholate(out) + glutarate(in) = glycocholate(in) + glutarate(out)

  Catalysed by GCAT, BAAT, SLC22A24mitochondrial matrix, peroxisome, inner side of the membrane, outer side of the membraneOne of 2 routes

• (5Z,8Z,11Z,14Z)-eicosatetraenoateReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. gamma-L-glutamyl-L-cysteine:glycine ligase (ADP-forming)
  3. transport of arachidonate and GSH (cytosol to extracellular)

  Catalysed by GCAT, GSSmitochondrial matrix, cytosol, extracellular spaceOne of 3 routes

• L-lysineReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. L-Arginine:glycine amidinotransferase
  3. L-ornithine(out) + L-lysine(in) = L-ornithine(in) + L-lysine(out)

  Catalysed by GCAT, GATM, SLC25A2, SLC25A15mitochondrial matrix, cytosol, inner side of the membrane, outer side of the membraneOne of 7 routes

• D-lysineReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. L-Arginine:glycine amidinotransferase
  3. D-lysine(in) + L-ornithine(out) = D-lysine(out) + L-ornithine(in)

  Catalysed by GCAT, GATM, SLC25A2mitochondrial matrix, cytosol, inner side of the membrane, outer side of the membraneOne of 2 routes

• putrescineReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. L-Arginine:glycine amidinotransferase
  3. L-ornithine carboxy-lyase (putrescine-forming)

  Catalysed by GCAT, GATM, ODC1mitochondrial matrix, cytosolOne of 4 routes

• D-arginineReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. L-Arginine:glycine amidinotransferase
  3. D-arginine(in) + L-ornithine(out) = D-arginine(out) + L-ornithine(in)

  Catalysed by GCAT, GATM, SLC25A2mitochondrial matrix, cytosol, inner side of the membrane, outer side of the membraneOne of 2 routes

• 4-methylthio-2-oxobutanoic acidReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. transport of glycine and methionine (cytosol to extracellular)
  3. L-Methionine:oxygen oxidoreductase (deaminating)

  Catalysed by GCAT, SLC7A5, IL4I1mitochondrial matrix, extracellular space, cytosol

• di-mu-sulfido-diiron(2+)Reach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. transport of cysteine and glycine (cytosol to extracellular)
  3. De novo synthesis of 2Fe2S

  Catalysed by GCAT, SLC3A2, SLC7A5, SLC7A8 and 7 moremitochondrial matrix, extracellular space, cytosol

• serotoninReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. gamma-L-glutamyl-L-cysteine:glycine ligase (ADP-forming)
  3. transport of GSH and serotonin (cytosol to extracellular)

  Catalysed by GCAT, GSS, SLCO1A2, SLCO1B3mitochondrial matrix, cytosol, extracellular spaceOne of 2 routes

• (S)-3-methyl-2-oxopentanoateReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. transport of glycine and isoleucine (cytosol to extracellular)
  3. L-isoleucine + 2-oxoglutarate = (S)-3-methyl-2-oxopentanoate + L-glutamate

  Catalysed by GCAT, SLC7A5, BCAT2, BCAT1mitochondrial matrix, extracellular space, cytosol

• D-cysteineReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. transport of alanine and glycine (cytosol to extracellular)
  3. D-cysteine(out) + L-alanine(in) = D-cysteine(in) + L-alanine(out)

  Catalysed by GCAT, SLC3A2, SLC7A5, SLC7A10mitochondrial matrix, extracellular space, cytosol, inner side of the membrane, outer side of the membraneOne of 2 routes

• D-serineReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. transport of alanine and glycine (cytosol to extracellular)
  3. D-serine(out) + L-alanine(in) = D-serine(in) + L-alanine(out)

  Catalysed by GCAT, SLC3A2, SLC7A5, SLC7A10mitochondrial matrix, extracellular space, cytosol, inner side of the membrane, outer side of the membraneOne of 10 routes

• 3-methyl-2-oxovaleric acidReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. transport of glycine and isoleucine (cytosol to extracellular)
  3. L-Leucine:2-oxoglutarate aminotransferase

  Catalysed by GCAT, SLC7A5, BCAT1mitochondrial matrix, extracellular space, cytosolOne of 2 routes

• 3-(4-hydroxyphenyl)pyruvateReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. transport of glycine and tyrosine (cytosol to extracellular)
  3. L-tyrosine:2-oxoglutarate aminotransferase

  Catalysed by GCAT, SLC7A5, GOT2, GOT1 and 1 moremitochondrial matrix, extracellular space, cytosolOne of 7 routes

• taurocholateReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. gamma-L-glutamyl-L-cysteine:glycine ligase (ADP-forming)
  3. solute carrier organic anion transporter (GSH)

  Catalysed by GCAT, GSS, SLCO1A2, SLCO1B3mitochondrial matrix, cytosol, extracellular spaceOne of 3 routes

• tert-butanolReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. gamma-L-glutamyl-L-cysteine + glycine + ATP = glutathione + ADP + phosphate + H(+)
  3. tert-butyl hydroperoxide + 2 glutathione = tert-butanol + glutathione disulfide + H2O

  Catalysed by GCAT, GSS, CLIC2, GPX1 and 3 moremitochondrial matrix

• gamma-Glu-AlaReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. gamma-L-glutamyl-L-cysteine:glycine ligase (ADP-forming)
  3. gamma-glutamyltransferase (alanine)

  Catalysed by GCAT, GSS, GGT1, GGT5 and 4 moremitochondrial matrix, cytosol, extracellular spaceOne of 2 routes

• taurineReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. bile acid-CoA: amino acid N-acyltransferase
  3. bile acid-CoA:amino acid N-acyltransferase (taurodeoxycholate)

  Catalysed by GCAT, BAATmitochondrial matrix, peroxisome, cytosolOne of 4 routes

• 3,3',5-triiodo-L-thyronineReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. gamma-L-glutamyl-L-cysteine:glycine ligase (ADP-forming)
  3. transport of GSH and triiodothyronine (cytosol to extracellular)

  Catalysed by GCAT, GSS, SLCO1A2, SLCO1B3mitochondrial matrix, cytosol, extracellular spaceOne of 2 routes

• (R)-methylmalonyl-CoAReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. bile acid-CoA: amino acid N-acyltransferase
  3. aldehyde dehydrogenase (2-methyl-3-oxopropanoate)

  Catalysed by GCAT, BAAT, ALDH6A1mitochondrial matrix, peroxisomeOne of 2 routes

• (S)-methylmalonyl-CoAReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. bile acid-CoA: amino acid N-acyltransferase
  3. (S)-methylmalonyl-CoA hydrolase

  Catalysed by GCAT, BAATmitochondrial matrix, peroxisome

• 3'-dephospho-CoAReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. bile acid-CoA: amino acid N-acyltransferase
  3. acid phosphatase 2, lysosomal (CoA)

  Catalysed by GCAT, BAAT, ACP2mitochondrial matrix, peroxisome, lysosomeOne of 2 routes

• (2E)-dodecenoyl-CoAReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. bile acid-CoA: amino acid N-acyltransferase
  3. (2E)-dodecenoyl-CoA formation

  Catalysed by GCAT, BAATmitochondrial matrix, peroxisomeOne of 3 routes

• 4,8,12-trimethyltridecanoyl-CoAReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. bile acid-CoA: amino acid N-acyltransferase
  3. propanoyl-CoA C-acyltransferase (3-ketopristanoyl-CoA)

  Catalysed by GCAT, BAAT, ACAA1, MYO5Bmitochondrial matrix, peroxisomeOne of 2 routes

• (6Z,9Z,12Z)-octadecatrienoyl-CoAReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. bile acid-CoA: amino acid N-acyltransferase
  3. long-chain-fatty-acid-CoA ligase (gamma-linolenate)

  Catalysed by GCAT, BAAT, SLC27A2, ACSL4 and 6 moremitochondrial matrix, peroxisome, cytosolOne of 7 routes

• benzoyl-CoAReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. Hippurate (Benzoylglycinate) Formation
  3. benzoyl-CoA + glycine = N-benzoylglycine + CoA + H(+)

  Catalysed by GCAT, GLYATmitochondrial matrix

• butanoyl-CoAReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. bile acid-CoA: amino acid N-acyltransferase
  3. Butanoate:CoA ligase (AMP-forming)

  Catalysed by GCAT, BAAT, ACSM4, ACSM1 and 4 moremitochondrial matrix, peroxisome, cytosolOne of 9 routes

• dodecanoyl-CoAReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. bile acid-CoA: amino acid N-acyltransferase
  3. long-chain-fatty-acid-CoA ligase (lauric acid)

  Catalysed by GCAT, BAAT, SLC27A2, ACSL4 and 6 moremitochondrial matrix, peroxisome, cytosolOne of 10 routes

• glutaryl-CoAReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. bile acid-CoA: amino acid N-acyltransferase
  3. Production of Glutaryl Carnitine

  Catalysed by GCAT, BAAT, CPT1A, CPT1C and 1 moremitochondrial matrix, peroxisome, cytosolOne of 2 routes

• hexadecanoyl-CoAReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. hexadecanoyl-CoA + glycine = N-hexadecanoylglycine + CoA + H(+)
  3. hexadecanoyl-CoA + glycine = N-hexadecanoylglycine + CoA + H(+)

  Catalysed by GCAT, GLYATL3mitochondrial matrix

• eicosanoyl-CoAReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. bile acid-CoA: amino acid N-acyltransferase
  3. long-chain-fatty-acid-CoA ligase (eicosanoate)

  Catalysed by GCAT, BAAT, SLC27A2, ACSL4 and 6 moremitochondrial matrix, peroxisome, cytosolOne of 12 routes

• itaconyl-CoAReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. bile acid-CoA: amino acid N-acyltransferase
  3. Itaconate:CoA ligase (GDP-forming)

  Catalysed by GCAT, BAAT, SUCLG1, SUCLG2 and 1 moremitochondrial matrix, peroxisomeOne of 2 routes

• (9Z,12Z)-octadecadienoyl-CoAReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. bile acid-CoA: amino acid N-acyltransferase
  3. long-chain-fatty-acid-CoA ligase ((6Z,9Z)-octadecadienoic acid)

  Catalysed by GCAT, BAAT, SLC27A2, ACSL4 and 6 moremitochondrial matrix, peroxisome, cytosolOne of 14 routes

• tetradecanoyl-CoAReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. bile acid-CoA: amino acid N-acyltransferase
  3. long-chain-fatty-acid-CoA ligase (myristic acid)

  Catalysed by GCAT, BAAT, SLC27A2, ACSL4 and 6 moremitochondrial matrix, peroxisome, cytosolOne of 12 routes

• octanoyl-CoAReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. bile acid-CoA: amino acid N-acyltransferase
  3. Octanoyl-CoA:L-carnitine O-octanoyltransferase

  Catalysed by GCAT, BAAT, CROTmitochondrial matrix, peroxisomeOne of 21 routes

• phytanoyl-CoAReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. bile acid-CoA: amino acid N-acyltransferase
  3. phytanate-CoA ligase

  Catalysed by GCAT, BAATmitochondrial matrix, peroxisome, cytosolOne of 5 routes

• octadecanoyl-CoAReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. bile acid-CoA: amino acid N-acyltransferase
  3. long-chain-fatty-acid-CoA ligase (stearate)

  Catalysed by GCAT, BAAT, SLC27A2, ACSL4 and 5 moremitochondrial matrix, peroxisome, cytosolOne of 15 routes

• prostaglandin E1Reach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. gamma-L-glutamyl-L-cysteine:glycine ligase (ADP-forming)
  3. transport of GSH and prostaglandin E1 (cytosol to extracellular)

  Catalysed by GCAT, GSS, SLCO2A1mitochondrial matrix, cytosol, extracellular spaceOne of 2 routes

• prostaglandin I2Reach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. gamma-L-glutamyl-L-cysteine:glycine ligase (ADP-forming)
  3. transport of GSH and prostaglandin I2 (cytosol to extracellular)

  Catalysed by GCAT, GSS, SLCO2A1mitochondrial matrix, cytosol, extracellular spaceOne of 2 routes

• prostaglandin F2alphaReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. gamma-L-glutamyl-L-cysteine:glycine ligase (ADP-forming)
  3. transport of GSH and prostaglandin F2alpha (cytosol to extracellular)

  Catalysed by GCAT, GSS, SLCO2A1mitochondrial matrix, cytosol, extracellular spaceOne of 2 routes

• prostaglandin H2Reach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. gamma-L-glutamyl-L-cysteine:glycine ligase (ADP-forming)
  3. transport of GSH and prostaglandin H2 (cytosol to extracellular)

  Catalysed by GCAT, GSS, SLCO2A1mitochondrial matrix, cytosol, extracellular spaceOne of 2 routes

• prostaglandin D2Reach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. gamma-L-glutamyl-L-cysteine:glycine ligase (ADP-forming)
  3. transport of GSH and prostaglandin D2 (cytosol to extracellular)

  Catalysed by GCAT, GSS, SLCO2A1mitochondrial matrix, cytosol, extracellular spaceOne of 2 routes

• (15S)-hydroxy-(5Z,8Z,11Z,13E)-eicosatetraenoateReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. gamma-L-glutamyl-L-cysteine:glycine ligase (ADP-forming)
  3. Glutathione: 15-HPETE oxidoreductase

  Catalysed by GCAT, GSS, GPX5, GPX1 and 7 moremitochondrial matrix, cytosolOne of 2 routes

• D-alanineReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. transport of alanine and glycine (cytosol to extracellular)
  3. D-alanine(out) + L-alanine(in) = D-alanine(in) + L-alanine(out)

  Catalysed by GCAT, SLC3A2, SLC7A5, SLC7A10mitochondrial matrix, extracellular space, cytosol, inner side of the membrane, outer side of the membraneOne of 2 routes

• 7,8-dihydrofolateReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. 5,10-Methylenetetrahydrofolate:glycine hydroxymethyltransferase
  3. 5,6,7,8-tetrahydrofolate:NAD+ oxidoreductase

  Catalysed by GCAT, SHMT1, DHFR, DHFR2mitochondrial matrix, cytosolOne of 3 routes