L-2-amino-3-oxobutanoic acid

Three steps

• N-decanoyl-(2S)-hydroxyglycineReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. glycine + 2-oxoglutarate = glyoxylate + L-glutamate
  3. N-decanoyl-(2S)-hydroxyglycine = decanamide + glyoxylate

  Catalysed by GCAT, AADAT, PAMmitochondrial matrixOne of 2 routes

• N-dodecanoyl-(2S)-hydroxyglycineReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. glycine + 2-oxoglutarate = glyoxylate + L-glutamate
  3. N-dodecanoyl-(2S)-hydroxyglycine = dodecanamide + glyoxylate

  Catalysed by GCAT, AADAT, PAMmitochondrial matrixOne of 2 routes

• N-tetradecanoyl-(2S)-hydroxyglycineReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. glycine + 2-oxoglutarate = glyoxylate + L-glutamate
  3. N-tetradecanoyl-(2S)-hydroxyglycine = tetradecamide + glyoxylate

  Catalysed by GCAT, AADAT, PAMmitochondrial matrixOne of 2 routes

• N-(9Z-octadecenoyl)-(2S)-hydroxyglycineReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. glycine + 2-oxoglutarate = glyoxylate + L-glutamate
  3. N-(9Z-octadecenoyl)-(2S)-hydroxyglycine = (9Z)-octadecenamide + glyoxylate

  Catalysed by GCAT, AADAT, PAMmitochondrial matrixOne of 2 routes

• N-(9Z,12Z,15Z)-octadecatrienoyl-(2S)-hydroxyglycineReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. glycine + 2-oxoglutarate = glyoxylate + L-glutamate
  3. N-(9Z,12Z,15Z)-octadecatrienoyl-(2S)-hydroxyglycine = (9Z,12Z,15Z)-octadecatrienamide + glyoxylate

  Catalysed by GCAT, AADAT, PAMmitochondrial matrix

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

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

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

  Catalysed by GCAT, GATM, SLC25A2mitochondrial matrix, cytosol, inner side of the membrane, outer side of the membraneOne of 4 routes

• L-homocarnosineReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. transport of glycine and histidine (cytosol to extracellular)
  3. Formation of homocarnosine

  Catalysed by GCAT, SLC7A6, CARNS1mitochondrial matrix, extracellular space, cytosol

• 2,3-didehydrophenylalanineReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. glycyldehydrophenylalanine + H2O = 2,3-didehydrophenylalanine + glycine
  3. glycyldehydrophenylalanine + H2O = 2,3-didehydrophenylalanine + glycine

  Catalysed by GCAT, DPEP1mitochondrial matrix

• S-adenosylglutathioneReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. gamma-L-glutamyl-L-cysteine + glycine + ATP = glutathione + ADP + phosphate + H(+)
  3. apo-[alkylcobalamin reductase] + adenosylcob(III)alamin + glutathione = S-adenosylglutathione + cob(I)alamin-[alkylcobalamin reductase] + H(+)

  Catalysed by GCAT, GSS, MMACHCmitochondrial matrix

• N(2)-formyl-N(1)-(5-phospho-beta-D-ribosyl)glycinamideReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. 5-phospho-beta-D-ribosylamine + glycine + ATP = N(1)-(5-phospho-beta-D-ribosyl)glycinamide + ADP + phosphate + H(+)
  3. N(1)-(5-phospho-beta-D-ribosyl)glycinamide + (6R)-10-formyltetrahydrofolate = N(2)-formyl-N(1)-(5-phospho-beta-D-ribosyl)glycinamide + (6S)-5,6,7,8-tetrahydrofolate + H(+)

  Catalysed by GCAT, GARTmitochondrial matrix

• 2-formamido-N(1)-(5-O-phospho-beta-D-ribosyl)acetamidineReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. transport of glutamine and glycine (cytosol to extracellular)
  3. N(2)-formyl-N(1)-(5-phospho-beta-D-ribosyl)glycinamide + L-glutamine + ATP + H2O = 2-formamido-N(1)-(5-O-phospho-beta-D-ribosyl)acetamidine + L-glutamate + ADP + phosphate + H(+)

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

• kinetensinReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. transport of glycine and phenylalanine (cytosol to extracellular)
  3. Chymase

  Catalysed by GCAT, SLC7A5, CMA1mitochondrial matrix, extracellular space, cytosolOne of 3 routes

• (7Z)-octadecenoyl-CoAReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. bile acid-CoA: amino acid N-acyltransferase
  3. carnitine O-palmitoyltransferase ((7Z)-octadecenoyl-CoA)

  Catalysed by GCAT, BAAT, CPT1A, CPT1C and 1 moremitochondrial matrix, peroxisome, cytosolOne of 3 routes

• acetaldehydeReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. transport of glycine and threonine (cytosol to extracellular)
  3. L-threonine acetaldehyde-lyase (glycine-forming)

  Catalysed by GCAT, SLC3A2, SLC7A5, SLC7A10mitochondrial matrix, extracellular space, cytosol

• CHEBI:15345Reach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. bile acid-CoA: amino acid N-acyltransferase
  3. Acetoacetate:CoA ligase (AMP-forming)

  Catalysed by GCAT, BAAT, AACSmitochondrial matrix, peroxisome, cytosolOne of 2 routes

• cholineReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. bile acid-CoA: amino acid N-acyltransferase
  3. Acetyl-CoA:choline O-acetyltransferase

  Catalysed by GCAT, BAAT, CHATmitochondrial matrix, peroxisome, nucleusOne of 2 routes

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

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

• pyruvateReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. glycine:oxygen oxidoreductase (deaminating)
  3. L-Alanine:glyoxylate aminotransferase

  Catalysed by GCAT, DAO, AGXT, AGXT2mitochondrial matrix, peroxisomeOne of 20 routes

• 4a-hydroxytetrahydrobiopterinReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. transport of glycine and tryptophan (cytosol to extracellular)
  3. L-tryptophan,tetrahydrobiopterin:oxygen oxidoreductase(5-hydroxylating)

  Catalysed by GCAT, SLC7A5, TPH1, TPH2mitochondrial matrix, extracellular space, cytosolOne of 3 routes

• CHEBI:15380Reach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. bile acid-CoA: amino acid N-acyltransferase
  3. 5-aminolevulinate synthase (2-amino-3-oxoadipate)

  Catalysed by GCAT, BAAT, ALAS1, CPNE7mitochondrial matrix, peroxisomeOne of 8 routes

• CHEBI:15477Reach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. bile acid-CoA: amino acid N-acyltransferase
  3. glycine N-acyltransferase (2-methylbutyryl-CoA)

  Catalysed by GCAT, BAAT, GLYAT, GLYATL2 and 1 moremitochondrial matrix, peroxisomeOne of 3 routes

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

  Catalysed by GCAT, BAAT, CPT1A, CPT1Bmitochondrial matrix, peroxisome, cytosolOne of 2 routes

• isobutyryl-CoAReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. bile acid-CoA: amino acid N-acyltransferase
  3. acetyl-CoA C-acyltransferase (3-oxo-4-methyl-pentanoyl-CoA)

  Catalysed by GCAT, BAAT, HADHA, ACAA2 and 1 moremitochondrial matrix, peroxisomeOne of 3 routes

• 3-methylbut-2-enoyl-CoAReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. bile acid-CoA: amino acid N-acyltransferase
  3. glycine-N-acyltransferase (3-methylcrotonoylglycine)

  Catalysed by GCAT, BAAT, GLYATmitochondrial matrix, peroxisome

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

  Catalysed by GCAT, BAAT, GLYAT, GLYATL2 and 1 moremitochondrial matrix, peroxisomeOne of 3 routes

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

  Catalysed by GCAT, BAAT, SLC27A2, ACSL4 and 6 moremitochondrial matrix, peroxisome, cytosolOne of 11 routes

• CHEBI:15519Reach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. bile acid-CoA: amino acid N-acyltransferase
  3. propanoyl-CoA C-acyltransferase

  Catalysed by GCAT, BAAT, ACAA1, SCP2 and 1 moremitochondrial matrix, peroxisomeOne of 2 routes

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

  Catalysed by GCAT, BAAT, SLC27A2, ACSL4 and 6 moremitochondrial matrix, peroxisome, cytosolOne of 11 routes

• oleoyl-CoAReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. bile acid-CoA: amino acid N-acyltransferase
  3. Transport of octadecenoyl-CoA into Mitochondrial Matrix

  Catalysed by GCAT, BAAT, CPT1A, CPT1C and 1 moremitochondrial matrix, peroxisome, cytosolOne of 11 routes

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

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

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

  Catalysed by GCAT, BAAT, ACSS3, ACSS2 and 1 moremitochondrial matrix, peroxisome, cytosolOne of 43 routes

• (S)-malateReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. glycine:oxygen oxidoreductase (deaminating)
  3. acetyl-CoA[m] + glyoxalate[m] + H2O[m] = CoA[m] + H+[m] + malate[m]

  Catalysed by GCAT, DAO, CLYBLmitochondrial matrix, peroxisomeOne of 2 routes

• (6R)-5,10-methylene-5,6,7,8-tetrahydrofolateReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. (6R)-5,10-methylene-5,6,7,8-tetrahydrofolate + glycine + H2O = (6S)-5,6,7,8-tetrahydrofolate + L-serine
  3. (6R)-5,10-methylene-5,6,7,8-tetrahydrofolate + glycine + H2O = (6S)-5,6,7,8-tetrahydrofolate + L-serine

  Catalysed by GCAT, SHMT1, SHMT2mitochondrial matrixOne of 2 routes

• 10-formyltetrahydrofolic acidReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. 5,10-Methylenetetrahydrofolate:glycine hydroxymethyltransferase
  3. Formate:tetrahydrofolate ligase (ADP-forming)

  Catalysed by GCAT, SHMT1, MTHFD1mitochondrial matrix, cytosolOne of 3 routes

• 5-methyltetrahydrofolic acidReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. 5,10-Methylenetetrahydrofolate:glycine hydroxymethyltransferase
  3. histamine N-methyltransferase (SAM)

  Catalysed by GCAT, SHMT1, HNMTmitochondrial matrix, cytosol

• (4aS,6R)-4a-hydroxy-L-erythro-5,6,7,8-tetrahydrobiopterinReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. transport of glycine 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 GCAT, SLC7A5, TPH1, TPH2mitochondrial matrix, extracellular space, cytosolOne of 3 routes

• CHEBI:15650Reach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. bile acid-CoA: amino acid N-acyltransferase
  3. N-acetyl-LTE4 to leukotriene E4 conversion

  Catalysed by GCAT, BAAT, ZDHHC11B, NAA10 and 30 moremitochondrial matrix, peroxisome, cytosolOne of 6 routes

• trimethylenediamineReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. transport of glycine and lysine (cytosol to extracellular)
  3. deoxyhypusine synthase (1,3-diaminopropane)

  Catalysed by GCAT, SLC7A6, DHPSmitochondrial matrix, extracellular space, cytosol

• CHEBI:15727Reach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. bile acid-CoA: amino acid N-acyltransferase
  3. N-alpha-acetyltransferase 10, NatA catalytic subunit (acetylcarnosine)

  Catalysed by GCAT, BAAT, NAA10mitochondrial matrix, peroxisome, cytosolOne of 2 routes

• CHEBI:15746Reach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. bile acid-CoA: amino acid N-acyltransferase
  3. diamine N-acetyltransferase (N1-acetylspermine)

  Catalysed by GCAT, BAAT, SAT1, SAT2mitochondrial matrix, peroxisome, cytosolOne of 2 routes

• CHEBI:15765Reach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. transport of glycine and tyrosine (cytosol to extracellular)
  3. L-Tyrosine,tetrahydrobiopterin:oxygen oxidoreductase (3-hydroxylating)

  Catalysed by GCAT, SLC7A5, THmitochondrial matrix, extracellular space, cytosolOne of 5 routes

• bromideReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. gamma-L-glutamyl-L-cysteine:glycine ligase (ADP-forming)
  3. 2-bromoacetaldehyde:glutathione S-(formylmethyl)transferase

  Catalysed by GCAT, GSS, MGST3, GSTA4 and 15 moremitochondrial matrix, cytosolOne of 2 routes

• alpha-D-glucosamine 6-phosphateReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. transport of glutamine and glycine (cytosol to extracellular)
  3. L-glutamine:D-fructose-6-phosphate isomerase (deaminating)

  Catalysed by GCAT, SLC7A5, GFPT2, GFPT1mitochondrial matrix, extracellular space, cytosol

• thioredoxin dithiolReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. glycine:tRNA(Gly) ligase (AMP-forming)
  3. glycyl-tRNA(gly) phosphorylation

  Catalysed by GCAT, GARS1mitochondrial matrix, cytosolOne of 2 routes

• a halide anionReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. gamma-L-glutamyl-L-cysteine + glycine + ATP = glutathione + ADP + phosphate + H(+)
  3. RX + glutathione = an S-substituted glutathione + a halide anion + H(+)

  Catalysed by GCAT, GSS, GSTT4, GSTA4 and 21 moremitochondrial matrix

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

  Catalysed by GCAT, SLC6A9, SLC6A14, SLC26A6mitochondrial matrix, extracellular space, cytosol

• ureaReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. transport of arginine and glycine (cytosol to extracellular)
  3. L-Arginine amidinohydrolase

  Catalysed by GCAT, SLC7A6, ARG1mitochondrial matrix, extracellular space, cytosolOne of 3 routes

• L-cystineReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. transport of alanine and glycine (cytosol to extracellular)
  3. Transport of L-Cystine (L-Alanine antiport) by B0, +At Transporter

  Catalysed by GCAT, SLC3A2, SLC7A5, SLC7A10 and 2 moremitochondrial matrix, extracellular space, cytosolOne of 8 routes

• 11-cis-retinolReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. bile acid-CoA: amino acid N-acyltransferase
  3. Palmitoyl-CoA:retinol O-acyltransferase

  Catalysed by GCAT, BAAT, DGAT1, LRAT and 3 moremitochondrial matrix, peroxisome, cytosol