L-2-amino-3-oxobutanoic acid

Three steps

• (R)-3-aminoisobutyric acidReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. transport of alanine and glycine (cytosol to extracellular)
  3. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase

  Catalysed by GCAT, SLC3A2, SLC7A5, SLC7A10 and 1 moremitochondrial matrix, extracellular space, cytosol

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

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

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

  Catalysed by GCAT, SLC6A9, SLC6A14, SLC26A4mitochondrial matrix, extracellular space, cytosolOne of 3 routes

• oxaloacetateReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. bile acid-CoA: amino acid N-acyltransferase
  3. ATP citrate lyase (citrate)

  Catalysed by GCAT, BAAT, ACLYmitochondrial matrix, peroxisome, cytosol

• nitric oxideReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. transport of arginine and glycine (cytosol to extracellular)
  3. L-Arginine, NADPH:Oxygen Oxidoreductase (Nitric-Oxide-Forming)

  Catalysed by GCAT, SLC7A6, NOS3, NOS1 and 1 moremitochondrial matrix, extracellular space, cytosolOne of 2 routes

• N(2)-acetyl-L-ornithineReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. L-Arginine:glycine amidinotransferase
  3. N2-Acetyl-L-ornithine amidohydrolase

  Catalysed by GCAT, GATM, ACY1mitochondrial matrix, cytosol

• 2-phenylacetamideReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. transport of glycine and phenylalanine (cytosol to extracellular)
  3. L-phenylalanine:oxygen 2-oxidoreductase (decarboxylating)

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

• D-alanyl-D-alanineReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. transport of D-alanine and glycine (cytosol to extracellular)
  3. Formation of Alanylalanine

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

• CHEBI:16633Reach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. transport of alanine and glycine (cytosol to extracellular)
  3. L-selenocysteine selenide-lyase (L-alanine-forming)

  Catalysed by GCAT, SLC3A2, SLC7A5, SLC7A10 and 1 moremitochondrial matrix, extracellular space, cytosol

• Met-tRNA(Met)Reach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. transport of glycine and methionine (cytosol to extracellular)
  3. L-Methionine:tRNAMet ligase (AMP-forming)

  Catalysed by GCAT, SLC7A5, MARS1mitochondrial matrix, extracellular space, cytosol

• 4-methylsulfanyl-2-oxobutanoateReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. transport of glycine and methionine (cytosol to extracellular)
  3. glyoxylate + L-methionine = 4-methylsulfanyl-2-oxobutanoate + glycine

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

• N-[(S)-lactoyl]-L-phenylalanineReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. transport of glycine and phenylalanine (cytosol to extracellular)
  3. (S)-lactate + L-phenylalanine = N-[(S)-lactoyl]-L-phenylalanine + H2O

  Catalysed by GCAT, SLC7A5, CNDP2mitochondrial matrix, extracellular space, cytosol

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

  Catalysed by GCAT, SLC3A2, SLC7A5, SLC7A10 and 2 moremitochondrial matrix, extracellular space, cytosolOne of 5 routes

• tryptamineReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. transport of glycine and tryptophan (cytosol to extracellular)
  3. L-tryptophan decarboxy-lyase

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

• 2-oxoglutaramateReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. transport of glutamine and glycine (cytosol to extracellular)
  3. L-Glutamine:pyruvate aminotransferase

  Catalysed by GCAT, SLC7A5, KYAT1mitochondrial matrix, extracellular space, cytosolOne of 3 routes

• formaldehydeReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. S-Adenosyl-L-methionine:glycine N-methyltransferase
  3. sarcosine:oxygen oxidoreductase (demethylating)

  Catalysed by GCAT, GNMT, PIPOX, SARDHmitochondrial matrix, cytosol, peroxisomeOne of 2 routes

• creatineReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. L-Arginine:glycine amidinotransferase
  3. S-Adenosyl-L-methionine:guanidinoacetate N-methyltransferase

  Catalysed by GCAT, GATM, GAMTmitochondrial matrix, cytosolOne of 2 routes

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

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

• oxalic acidReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. glycine:oxygen oxidoreductase (deaminating)
  3. Glyoxylate:oxygen oxidoreductase

  Catalysed by GCAT, DAO, HAO1mitochondrial matrix, peroxisomeOne of 3 routes

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

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

• CHEBI:17208Reach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. gamma-L-glutamyl-L-cysteine:glycine ligase (ADP-forming)
  3. glutathione:dehydroascorbate oxidoreductase

  Catalysed by GCAT, GSSmitochondrial matrix, cytosol

• homocysteineReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. gamma-L-glutamyl-L-cysteine:glycine ligase (ADP-forming)
  3. Formation of Homocystine

  Catalysed by GCAT, GSSmitochondrial matrix, cytosol

• L-glutamic 5-semialdehydeReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. L-Arginine:glycine amidinotransferase
  3. L-Ornithine:2-oxo-acid aminotransferase

  Catalysed by GCAT, GATM, OATmitochondrial matrix, cytosol

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

  Catalysed by GCAT, BAAT, NAT2, NAT1mitochondrial matrix, peroxisome, cytosol

• CHEBI:17312Reach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. bile acid-CoA: amino acid N-acyltransferase
  3. N1,N12-diacetylspermine to N1-acetylspermine conversion

  Catalysed by GCAT, BAATmitochondrial matrix, peroxisome, cytosolOne of 2 routes

• sulfiteReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. gamma-L-glutamyl-L-cysteine + glycine + ATP = glutathione + ADP + phosphate + H(+)
  3. thiosulfate + 2 glutathione = glutathione disulfide + hydrogen sulfide + sulfite + 2 H(+)

  Catalysed by GCAT, GSS, TSTD1mitochondrial matrixOne of 2 routes

• CHEBI:17380Reach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. transport of glycine and tryptophan (cytosol to extracellular)
  3. Transport of 3-Hydroxy-L-Kynurenine

  Catalysed by GCAT, SLC7A5mitochondrial matrix, extracellular space, cytosolOne of 2 routes

• (S)-1-pyrroline-5-carboxylateReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. transport of glycine and proline (cytosol to extracellular)
  3. L-proline:(acceptor) oxidoreductase

  Catalysed by GCAT, SLC7A5, SLC7A6, PRODH2mitochondrial matrix, extracellular space, cytosolOne of 2 routes

• agmatineReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. transport of arginine and glycine (cytosol to extracellular)
  3. L-arginine carboxy-lyase (agmatine-forming)

  Catalysed by GCAT, SLC7A6mitochondrial matrix, extracellular space, cytosol

• AH2Reach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. transport of alanine and glycine (cytosol to extracellular)
  3. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O

  Catalysed by GCAT, SLC3A2, SLC7A5, SLC7A10 and 1 moremitochondrial matrix, extracellular space, cytosolOne of 4 routes

• indole-3-pyruvateReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. transport of glycine and tryptophan (cytosol to extracellular)
  3. L-tryptophan + O2 + H2O = indole-3-pyruvate + H2O2 + NH4(+)

  Catalysed by GCAT, SLC7A5, IL4I1mitochondrial matrix, extracellular space, cytosolOne of 3 routes

• (7Z,10Z,13Z)-hexadecatrienoyl-CoAReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. bile acid-CoA: amino acid N-acyltransferase
  3. Fatty Acid Beta Oxidation (C18:3->C16:3), Mitochondrial

  Catalysed by GCAT, BAAT, HADHA, ACADVL and 1 moremitochondrial matrix, peroxisome

• CHEBI:17724Reach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. S-Adenosyl-L-methionine:glycine N-methyltransferase
  3. dimethylglycine dehydrogenase (formaldehyde)

  Catalysed by GCAT, GNMT, PDPR, DMGDHmitochondrial matrix, cytosol

• Ala-tRNA(Ala)Reach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. transport of alanine and glycine (cytosol to extracellular)
  3. L-Alanine:tRNA(Ala) ligase (AMP-forming)

  Catalysed by GCAT, SLC3A2, SLC7A5, SLC7A10 and 1 moremitochondrial matrix, extracellular space, cytosol

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

  Catalysed by GCAT, SLC6A9, SLC6A14, SLC6A12mitochondrial matrix, extracellular space, cytosolOne of 3 routes

• trans-urocanateReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. transport of glycine and histidine (cytosol to extracellular)
  3. L-histidine ammonia-lyase (urocanate-forming)

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

• urateReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. gamma-L-glutamyl-L-cysteine + glycine + ATP = glutathione + ADP + phosphate + H(+)
  3. urate(out) + glutathione(in) = urate(in) + glutathione(out)

  Catalysed by GCAT, GSS, SLC22A13mitochondrial matrix, inner side of the membrane, outer side of the membrane

• 5-hydroxy-L-tryptophanReach 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, cytosol

• methylarsonous acidReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. gamma-L-glutamyl-L-cysteine + glycine + ATP = glutathione + ADP + phosphate + H(+)
  3. methylarsonate + 2 glutathione + H(+) = methylarsonous acid + glutathione disulfide + H2O

  Catalysed by GCAT, GSS, GSTO1, GSTO2mitochondrial matrix

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

  Catalysed by GCAT, SLC7A5, BCAT2mitochondrial matrix, extracellular space, cytosolOne of 5 routes

• CHEBI:17927Reach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. bile acid-CoA: amino acid N-acyltransferase
  3. N1,N8-diacetylspermidine to N1-acetylspermidine conversion

  Catalysed by GCAT, BAATmitochondrial matrix, peroxisome, cytosolOne of 2 routes

• 3-phenylpyruvateReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. transport of glycine and phenylalanine (cytosol to extracellular)
  3. Phenylalanine Transaminase, Mitochondrial

  Catalysed by GCAT, SLC7A5, GOT2, TATmitochondrial matrix, extracellular space, cytosolOne of 6 routes

• CHEBI:18095Reach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. transport of chloride and glycine (cytosol to extracellular)
  3. transport of trans-4-hydroxy-L-proline (cytosol to extracellular)

  Catalysed by GCAT, SLC6A9, SLC6A14, CLTRN and 1 moremitochondrial matrix, extracellular space, cytosolOne of 5 routes

• N-carbamoyl-beta-alanineReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. transport of chloride and glycine (cytosol to extracellular)
  3. Transport of 3-Ureidopropionate

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

• N(2)-formyl-N(1)-(5-phospho-D-ribosyl)glycinamideReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. 5-Phospho-D-ribosylamine:glycine ligase (ADP-forming)
  3. 10-formyltetrahydrofolate:5'-phosphoribosylglycinamide formyltransferase

  Catalysed by GCAT, GARTmitochondrial matrix, cytosol

• arsenic triglutathioneReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. gamma-L-glutamyl-L-cysteine + glycine + ATP = glutathione + ADP + phosphate + H(+)
  3. arsenic triglutathione + 3 [thioredoxin]-dithiol + 3 S-adenosyl-L-methionine = trimethylarsine + 3 [thioredoxin]-disulfide + 3 glutathione + 3 S-adenosyl-L-homocysteine + 3 H(+)

  Catalysed by GCAT, GSS, AS3MTmitochondrial matrixOne of 3 routes

• 2-phenylethylamineReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. transport of glycine and phenylalanine (cytosol to extracellular)
  3. L-phenylalanine carboxy-lyase (phenylethylamine-forming)

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

• 2-formamido-N(1)-(5-phospho-D-ribosyl)acetamidineReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. transport of glutamine and glycine (cytosol to extracellular)
  3. 5'-Phosphoribosylformylglycinamide:L-glutamine amido-ligase (ADP-forming)

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

• gamma-L-glutamyl-(2S)-2-aminobutanoateReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  3. (2S)-2-aminobutanoate + L-glutamate + ATP = gamma-L-glutamyl-(2S)-2-aminobutanoate + ADP + phosphate + H(+)

  Catalysed by GCAT, AGXT2, GCLCmitochondrial matrix

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

  Catalysed by GCAT, SLC7A5, SLC7A8mitochondrial matrix, extracellular space, cytosol, inner side of the membrane, outer side of the membraneOne of 6 routes