L-2-amino-3-oxobutanoic acid

Three steps

• 5-formimidoyltetrahydrofolateReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. 5,10-Methylenetetrahydrofolate:glycine hydroxymethyltransferase
  3. 5-Formiminotetrahydrofolate:L-glutamate N-formiminotransferase

  Catalysed by GCAT, SHMT1, FTCDmitochondrial matrix, cytosolOne of 2 routes

• leukotriene A4Reach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. gamma-L-glutamyl-L-cysteine:glycine ligase (ADP-forming)
  3. transport of GSH and leukotriene A4 (cytosol to extracellular)

  Catalysed by GCAT, GSS, SLCO2A1mitochondrial matrix, cytosol, extracellular spaceOne of 3 routes

• 2-(N(omega)-L-arginino)succinateReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. transport of arginine and glycine (cytosol to extracellular)
  3. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)

  Catalysed by GCAT, SLC7A6, ASLmitochondrial matrix, extracellular space, cytosolOne of 4 routes

• (R)-S-lactoylglutathioneReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. gamma-L-glutamyl-L-cysteine:glycine ligase (ADP-forming)
  3. (R)-S-Lactoylglutathione methylglyoxal-lyase (isomerizing)

  Catalysed by GCAT, GSS, GLO1, GLOD4mitochondrial matrix, cytosolOne of 3 routes

• 4-guanidinobutanoateReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. transport of arginine and glycine (cytosol to extracellular)
  3. 4-aminobutanoate + L-arginine = 4-guanidinobutanoate + L-ornithine

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

• 2-oxoadipateReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. glycine + 2-oxoglutarate = glyoxylate + L-glutamate
  3. L-2-aminoadipate + glyoxylate = 2-oxoadipate + glycine

  Catalysed by GCAT, AADATmitochondrial matrix

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

  Catalysed by GCAT, SLC7A5, SLC7A8mitochondrial matrix, extracellular space, cytosol, inner side of the membrane, outer side of the membraneOne of 3 routes

• 3-guanidinopropanoateReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. transport of arginine and glycine (cytosol to extracellular)
  3. beta-alanine + L-arginine = 3-guanidinopropanoate + L-ornithine

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

• L-histidineReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. L-Arginine:glycine amidinotransferase
  3. L-histidine(in) + L-ornithine(out) + H(+)(in) = L-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 7 routes

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

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

• 2-oxo-3-sulfanylpropanoateReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. transport of cysteine and glycine (cytosol to extracellular)
  3. L-Cysteine:2-oxoglutarate aminotransferase

  Catalysed by GCAT, SLC3A2, SLC7A5, SLC7A8 and 2 moremitochondrial matrix, extracellular space, cytosolOne of 3 routes

• S-formylglutathioneReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. gamma-L-glutamyl-L-cysteine:glycine ligase (ADP-forming)
  3. alcohol dehydrogenase (formaldehyde)

  Catalysed by GCAT, GSS, ADH1C, ADH1A and 4 moremitochondrial matrix, cytosolOne of 2 routes

• sphing-4-enineReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. bile acid-CoA: amino acid N-acyltransferase
  3. acyl-CoA:sphingosine N-acyltransferase

  Catalysed by GCAT, BAAT, ASAH1, ACER2 and 2 moremitochondrial matrix, peroxisome, Golgi apparatusOne of 2 routes

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

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

  Catalysed by GCAT, BAAT, SAT1, SAT2mitochondrial matrix, peroxisome, cytosolOne of 3 routes

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

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

• L-threonineReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. transport of alanine and glycine (cytosol to extracellular)
  3. L-threonine(out) + L-alanine(in) = L-threonine(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 8 routes

• L-saccharopineReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. transport of glycine and lysine (cytosol to extracellular)
  3. N6-(L-1,3-Dicarboxypropyl)-L-lysine:NADP+ oxidoreductase (L-lysine-forming)

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

• L-kynurenineReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. transport of alanine and glycine (cytosol to extracellular)
  3. L-kynurenine + H2O = anthranilate + L-alanine + H(+)

  Catalysed by GCAT, SLC3A2, SLC7A5, SLC7A10 and 1 moremitochondrial matrix, extracellular space, cytosolOne of 5 routes

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

  Catalysed by GCAT, SLC6A9, SLC6A14, SLC6A6mitochondrial matrix, extracellular space, cytosolOne of 4 routes

• dimethylamineReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. N(omega),N(omega)-dimethyl-L-arginine + glyoxylate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + glycine
  3. Nomega,Nomega'-dimethyl-L-arginine dimethylamidohydrolase

  Catalysed by GCAT, AGXT2, DDAH1mitochondrial matrix, cytosolOne of 2 routes

• taurocyamineReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. transport of arginine and glycine (cytosol to extracellular)
  3. taurine + L-arginine = taurocyamine + L-ornithine

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

• L-glutamate 5-semialdehydeReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. L-Arginine:glycine amidinotransferase
  3. L-ornithine + 2-oxoglutarate = L-glutamate 5-semialdehyde + L-glutamate

  Catalysed by GCAT, GATM, OATmitochondrial matrix, cytosol

• 3-hydroxy-L-kynurenineReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. transport of alanine and glycine (cytosol to extracellular)
  3. 3-hydroxy-L-kynurenine + H2O = 3-hydroxyanthranilate + L-alanine + H(+)

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

• porphobilinogenReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. succinyl-CoA:glycine C-succinyltransferase (decarboxylating)
  3. 5-aminolevulinate hydro-lyase (adding 5-aminolevulinate and cyclizing

  Catalysed by GCAT, ALAS1, ALAS2, CPNE7 and 1 moremitochondrial matrix, cytosolOne of 2 routes

• L,L-cystathionineReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. bile acid-CoA: amino acid N-acyltransferase
  3. N-acetylglutamate synthase (N-acetyl-L-cystathionine)

  Catalysed by GCAT, BAAT, NAGSmitochondrial matrix, peroxisome

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

  Catalysed by GCAT, SLC3A2, SLC7A5, SLC7A8 and 2 moremitochondrial matrix, extracellular space, cytosolOne of 2 routes

• 6-amino-2-oxohexanoateReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. transport of glycine and lysine (cytosol to extracellular)
  3. L-lysine:oxygen 2-oxidoreductase (deaminating)

  Catalysed by GCAT, SLC7A6mitochondrial matrix, extracellular space, cytosol, peroxisome

• L-homocysteineReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. transport of glycine and methionine (cytosol to extracellular)
  3. S-methyl-L-methionine + L-homocysteine = 2 L-methionine + H(+)

  Catalysed by GCAT, SLC7A5, BHMT2mitochondrial matrix, extracellular space, cytosol

• carbamoyl phosphateReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. transport of glutamine and glycine (cytosol to extracellular)
  3. HCO3-:L-glutamine amido-ligase (ADP-forming, carbamate-phosphorylating)

  Catalysed by GCAT, SLC7A5, CADmitochondrial matrix, extracellular space, cytosolOne of 4 routes

• N,N-dimethylglycineReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. sarcosine + O2 + H2O = formaldehyde + glycine + H2O2
  3. (6S)-5,6,7,8-tetrahydrofolyl-(gamma-L-Glu)(n) + N,N-dimethylglycine + oxidized [electron-transfer flavoprotein] + H(+) = (6R)-5,10-methylenetetrahydrofolyl-(gamma-L-Glu)(n) + sarcosine + reduced [electron-transfer flavoprotein]

  Catalysed by GCAT, PIPOX, DMGDHmitochondrial matrix

• S-methyl-L-methionineReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. transport of glycine and methionine (cytosol to extracellular)
  3. S-methyl-L-methionine + L-homocysteine = 2 L-methionine + H(+)

  Catalysed by GCAT, SLC7A5, BHMT2mitochondrial matrix, extracellular space, cytosol

• glutathione disulfideReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. gamma-L-glutamyl-L-cysteine:glycine ligase (ADP-forming)
  3. Glutathione: 5-HPETE oxidoreductase

  Catalysed by GCAT, GSS, GPX5, GPX1 and 7 moremitochondrial matrix, cytosolOne of 35 routes

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

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

• an acyl-CoAReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. bile acid-CoA: amino acid N-acyltransferase
  3. acyl-CoA:sphingosine N-acyltransferase

  Catalysed by GCAT, BAAT, ASAH1, ACER2 and 2 moremitochondrial matrix, peroxisome, Golgi apparatusOne of 2 routes

• trans-4-hydroxy-L-prolineReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. transport of alanine and glycine (cytosol to extracellular)
  3. trans-4-hydroxy-L-proline(out) + L-alanine(in) + Na(+)(out) = trans-4-hydroxy-L-proline(in) + L-alanine(out) + Na(+)(in)

  Catalysed by GCAT, SLC3A2, SLC7A5, SLC7A10 and 1 moremitochondrial matrix, extracellular space, cytosol, inner side of the membrane, outer side of the membraneOne of 2 routes

• 5-oxo-L-prolineReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. gamma-L-glutamyl-L-cysteine + glycine + ATP = glutathione + ADP + phosphate + H(+)
  3. glutathione = L-cysteinylglycine + 5-oxo-L-proline

  Catalysed by GCAT, GSS, CHAC2, CHAC1mitochondrial matrix

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

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

• L-2-aminopentanoateReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. N(omega),N(omega)-dimethyl-L-arginine + glyoxylate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + glycine
  3. 2-oxopentanoate + N(omega),N(omega)-dimethyl-L-arginine = 5-(3,3-dimethylguanidino)-2-oxopentanoate + L-2-aminopentanoate

  Catalysed by GCAT, AGXT2mitochondrial matrix

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

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

• kynurenateReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. glycine + 2-oxoglutarate = glyoxylate + L-glutamate
  3. L-kynurenine + glyoxylate = kynurenate + glycine + H2O

  Catalysed by GCAT, AADAT, KYAT3mitochondrial matrix

• L-2-aminohexanoateReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. N(omega),N(omega)-dimethyl-L-arginine + glyoxylate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + glycine
  3. 2-oxohexanoate + N(omega),N(omega)-dimethyl-L-arginine = L-2-aminohexanoate + 5-(3,3-dimethylguanidino)-2-oxopentanoate

  Catalysed by GCAT, AGXT2mitochondrial matrix

• 5-amino-1-(5-phospho-beta-D-ribosyl)imidazole-4-carboxamideReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. 5,10-Methylenetetrahydrofolate:glycine hydroxymethyltransferase
  3. phosphoribosylaminoimidazolecarboxamide formyltransferase (10-formyl-THF)

  Catalysed by GCAT, SHMT1, ATICmitochondrial matrix, cytosol

• 5-guanidino-2-oxopentanoateReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. transport of arginine and glycine (cytosol to extracellular)
  3. L-arginine + O2 + H2O = 5-guanidino-2-oxopentanoate + H2O2 + NH4(+)

  Catalysed by GCAT, SLC7A6, IL4I1mitochondrial matrix, extracellular space, cytosol

• (3Z)-dodecenoyl-CoAReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. bile acid-CoA: amino acid N-acyltransferase
  3. acetyl-CoA C-acyltransferase (3-oxomyrist-5-enoyl-CoA)

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

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

  Catalysed by GCAT, SLC3A2, SLC7A5, SLC7A8 and 1 moremitochondrial matrix, extracellular space, cytosol

• N-formyl-L-kynurenineReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. transport of glycine and tryptophan (cytosol to extracellular)
  3. L-tryptophan + O2 = N-formyl-L-kynurenine

  Catalysed by GCAT, SLC7A5, IDO1, TDO2 and 1 moremitochondrial matrix, extracellular space, cytosol

• (25R)-3alpha,7alpha,12alpha-trihydroxy-5beta-cholestan-26-oyl-CoAReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. bile acid-CoA: amino acid N-acyltransferase
  3. 3alpha,7alpha,12alpha-trihydroxy-5beta-cholestanoate:CoA ligase (AMP-forming)

  Catalysed by GCAT, BAAT, SLC27A2, SLC27A5mitochondrial matrix, peroxisome, endoplasmic reticulumOne of 3 routes

• 5-phospho-beta-D-ribosylamineReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. transport of glutamine and glycine (cytosol to extracellular)
  3. phosphoribosyl pyrophosphate amidotransferase (glutamine)

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

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

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