L-2-amino-3-oxobutanoic acid

Three steps

• L-methionine (S)-S-oxideReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. transport of glycine and methionine (cytosol to extracellular)
  3. [thioredoxin]-disulfide + L-methionine + H2O = L-methionine (S)-S-oxide + [thioredoxin]-dithiol

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

• L-methionine (R)-S-oxideReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. transport of glycine and methionine (cytosol to extracellular)
  3. [thioredoxin]-disulfide + L-methionine + H2O = L-methionine (R)-S-oxide + [thioredoxin]-dithiol

  Catalysed by GCAT, SLC7A5, MSRB3, MSRB1 and 1 moremitochondrial matrix, extracellular space, cytosol

• 5-amino-2-oxopentanoateReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. L-Arginine:glycine amidinotransferase
  3. L-ornithine + pyruvate = 5-amino-2-oxopentanoate + L-alanine

  Catalysed by GCAT, GATM, AGXT2mitochondrial matrix, cytosolOne of 3 routes

• deoxycholoyl-CoAReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. bile acid-CoA: amino acid N-acyltransferase
  3. cholate-CoA ligase (3alpha,12alpha-dihydroxy-5beta-cholanate)

  Catalysed by GCAT, BAAT, SLC27A2, SLC27A5mitochondrial matrix, peroxisome, cytosolOne of 4 routes

• S-sulfanylglutathioneReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. gamma-L-glutamyl-L-cysteine:glycine ligase (ADP-forming)
  3. Sulfide:glutathione,quinone oxidoreductase

  Catalysed by GCAT, GSS, SQORmitochondrial matrix, cytosolOne of 5 routes

• methylamineReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. N(omega)-methyl-L-arginine + glyoxylate = 5-(3-methylguanidino)-2-oxopentanoate + glycine
  3. N(omega)-methyl-L-arginine + H2O = L-citrulline + methylamine

  Catalysed by GCAT, AGXT2, DDAH1mitochondrial matrix

• N-[(R)-4-phosphopantothenoyl]-L-cysteineReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. transport of cysteine and glycine (cytosol to extracellular)
  3. (R)-4'-phosphopantothenate:L-cysteine ligase

  Catalysed by GCAT, SLC3A2, SLC7A5, SLC7A8 and 1 moremitochondrial matrix, extracellular space, cytosolOne of 4 routes

• monodehydro-L-ascorbate radicalReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. (9Z)-octadecenoyl-CoA + glycine = N-(9Z-octadecenoyl)glycine + CoA + H(+)
  3. N-(9Z-octadecenoyl)glycine + 2 L-ascorbate + O2 = N-(9Z-octadecenoyl)-(2S)-hydroxyglycine + 2 monodehydro-L-ascorbate radical + H2O

  Catalysed by GCAT, GLYATL3, GLYATL2, PAMmitochondrial matrixOne of 5 routes

• (6R)-L-erythro-5,6,7,8-tetrahydrobiopterinReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. L-tyrosine + glyoxylate = 3-(4-hydroxyphenyl)pyruvate + glycine
  3. (6R)-L-erythro-5,6,7,8-tetrahydrobiopterin + L-phenylalanine + O2 = (4aS,6R)-4a-hydroxy-L-erythro-5,6,7,8-tetrahydrobiopterin + L-tyrosine

  Catalysed by GCAT, AADAT, PAHmitochondrial matrix

• 4-aminobutanoateReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. transport of chloride and glycine (cytosol to extracellular)
  3. transport of 4-aminobutyrate and chloride (cytosol to extracellular)

  Catalysed by GCAT, SLC6A9, SLC6A14, SLC6A12mitochondrial matrix, extracellular space, cytosolOne of 4 routes

• 3-iodo-L-tyrosineReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. transport of glycine and tyrosine (cytosol to extracellular)
  3. thyroid peroxidase (iodine)

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

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

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

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

  Catalysed by GCAT, BAAT, SULT2A1mitochondrial matrix, cytosolOne of 3 routes

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

• prostaglandin E2Reach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. gamma-L-glutamyl-L-cysteine:glycine ligase (ADP-forming)
  3. transport of GSH and prostaglandin E2 (cytosol to extracellular)

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

• 3-sulfino-L-alanineReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. transport of cysteine and glycine (cytosol to extracellular)
  3. L-Cysteine:oxygen oxidoreductase

  Catalysed by GCAT, SLC3A2, SLC7A5, SLC7A8 and 1 moremitochondrial matrix, extracellular space, cytosolOne of 2 routes

• decanoyl-CoAReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. bile acid-CoA: amino acid N-acyltransferase
  3. medium-chain acyl-CoA ligase (decanoic acid)

  Catalysed by GCAT, BAAT, ACSM4, ACSM1 and 4 moremitochondrial matrix, peroxisome, cytosolOne of 8 routes

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

  Catalysed by GCAT, BAAT, CPT1A, CPT1C and 1 moremitochondrial matrix, peroxisome, cytosolOne of 4 routes

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

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

• L-cysteinylglycineReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. gamma-L-glutamyl-L-cysteine:glycine ligase (ADP-forming)
  3. glutathione gamma-glutamylaminopeptidase

  Catalysed by GCAT, GSS, GGTLC3, GGT1 and 4 moremitochondrial matrix, cytosolOne of 8 routes

• (4R)-4-hydroxy-2-oxoglutarateReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. glycine + 2-oxoglutarate = glyoxylate + L-glutamate
  3. (4R)-4-hydroxy-2-oxoglutarate = glyoxylate + pyruvate

  Catalysed by GCAT, AADAT, HOGA1mitochondrial matrix

• 3-hydroxyisovaleryl-CoAReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. bile acid-CoA: amino acid N-acyltransferase
  3. (3-Hydroxyisovalerylcoa->3-Hydroxyisovalerylcarnitine)

  Catalysed by GCAT, BAAT, CPT1A, CPT1Bmitochondrial matrix, peroxisome, cytosol

• leukotriene D4Reach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. leukotriene C4 hydrolysis
  3. leukotriene C4 + H2O = leukotriene D4 + L-glutamate

  Catalysed by GCAT, GGT1, GGT5mitochondrial matrix, cytosol

• CHEBI:63544Reach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. bile acid-CoA: amino acid N-acyltransferase
  3. long-chain-fatty-acid-CoA ligase (adrenic acid)

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

• ubiquinol-10Reach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. gamma-L-glutamyl-L-cysteine + glycine + ATP = glutathione + ADP + phosphate + H(+)
  3. ubiquinone-10 + hydrogen sulfide + glutathione + H(+) = S-sulfanylglutathione + ubiquinol-10

  Catalysed by GCAT, GSS, SQORmitochondrial matrix

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

  Catalysed by GCAT, BAAT, SLC27A2, ACSL4 and 6 moremitochondrial matrix, peroxisome, cytosolOne of 5 routes

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

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

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

  Catalysed by GCAT, BAAT, SLC27A2, ACSL4 and 6 moremitochondrial matrix, peroxisome, cytosolOne of 9 routes

• N(5)-[(Z)-amino(hydroxyimino)methyl]ornithineReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. transport of arginine and glycine (cytosol to extracellular)
  3. L-arginine,NADPH:oxygen oxidoreductase (N-(omega)-hydroxyarginine-forming)

  Catalysed by GCAT, SLC7A6, NOS3, NOS1 and 1 moremitochondrial matrix, extracellular space, cytosol

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

  Catalysed by GCAT, AADAT, KYAT3mitochondrial matrix

• Mo-molybdopterinReach 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, cytosol

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

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

• (6Z,9Z,12Z,15Z)-octadecatetraenoyl-CoAReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. bile acid-CoA: amino acid N-acyltransferase
  3. long-chain-fatty-acid-CoA ligase (stearidonic acid)

  Catalysed by GCAT, BAAT, SLC27A2, ACSL4 and 6 moremitochondrial matrix, peroxisome, cytosolOne of 8 routes

• (4S)-4-hydroxy-2-oxoglutarateReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. glycine + 2-oxoglutarate = glyoxylate + L-glutamate
  3. (4S)-4-hydroxy-2-oxoglutarate = glyoxylate + pyruvate

  Catalysed by GCAT, AADAT, HOGA1mitochondrial matrix

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

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

• (5Z,8Z,11Z,14Z,17Z)-eicosapentaenoyl-CoAReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. bile acid-CoA: amino acid N-acyltransferase
  3. long-chain-fatty-acid-CoA ligase (EPA)

  Catalysed by GCAT, BAAT, SLC27A2, ACSL4 and 6 moremitochondrial matrix, peroxisome, cytosolOne of 9 routes

• (7Z,10Z,13Z,16Z,19Z)-docosapentaenoyl-CoAReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. bile acid-CoA: amino acid N-acyltransferase
  3. long-chain-fatty-acid-CoA ligase (DPA)

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

• (9Z,12Z,15Z)-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 (linolenate)

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

• (13Z)-docosenoyl-CoAReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. bile acid-CoA: amino acid N-acyltransferase
  3. long-chain-fatty-acid-CoA ligase (cis-erucic acid)

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

• (11Z)-eicosenoyl-CoAReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. bile acid-CoA: amino acid N-acyltransferase
  3. long-chain-fatty-acid-CoA ligase (cis-gondoic acid)

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

• (9Z,12Z,15Z,18Z,21Z)-tetracosapentaenoyl-CoAReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. bile acid-CoA: amino acid N-acyltransferase
  3. long-chain-fatty-acid-CoA ligase ((9Z,12Z,15Z,18Z,21Z)-TPA)

  Catalysed by GCAT, BAAT, SLC27A2, ACSL4 and 6 moremitochondrial matrix, peroxisome, cytosolOne of 5 routes

• (6Z,9Z,12Z,15Z,18Z,21Z)-tetracosahexaenoyl-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,12Z,15Z,18Z,21Z)-THA)

  Catalysed by GCAT, BAAT, SLC27A2, ACSL4 and 6 moremitochondrial matrix, peroxisome, cytosolOne of 4 routes

• (15Z)-tetracosenoyl-CoAReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. bile acid-CoA: amino acid N-acyltransferase
  3. long-chain-fatty-acid-CoA ligase (nervonic acid)

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

• (17Z)-hexacosenoyl-CoAReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. bile acid-CoA: amino acid N-acyltransferase
  3. long-chain-fatty-acid-CoA ligase (ximenic acid)

  Catalysed by GCAT, BAAT, SLC27A2, ACSL4 and 6 moremitochondrial matrix, peroxisome, cytosolOne of 4 routes

• (8Z,11Z,14Z)-eicosatrienoyl-CoAReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. bile acid-CoA: amino acid N-acyltransferase
  3. long-chain-fatty-acid-CoA ligase (dihomo-gamma-linolenate)

  Catalysed by GCAT, BAAT, SLC27A2, ACSL4 and 6 moremitochondrial matrix, peroxisome, cytosolOne of 5 routes

• (8Z,11Z,14Z,17Z)-eicosatetraenoyl-CoAReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. bile acid-CoA: amino acid N-acyltransferase
  3. long-chain-fatty-acid-CoA ligase (omega-3-arachidonic acid)

  Catalysed by GCAT, BAAT, SLC27A2, ACSL4 and 6 moremitochondrial matrix, peroxisome, cytosolOne of 5 routes

• (4Z,7Z,10Z,13Z,16Z,19Z)-docosahexaenoyl-CoAReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. bile acid-CoA: amino acid N-acyltransferase
  3. long-chain-fatty-acid-CoA ligase (DHA)

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

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

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

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

  Catalysed by GCAT, BAAT, SLC27A2, ACSL4 and 6 moremitochondrial matrix, peroxisome, cytosolOne of 5 routes

• (11Z,14Z,17Z)-eicosatrienoyl-CoAReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. bile acid-CoA: amino acid N-acyltransferase
  3. long-chain-fatty-acid-CoA ligase ((11Z,14Z,17Z)-eicosatrienoic acid)

  Catalysed by GCAT, BAAT, SLC27A2, ACSL4 and 6 moremitochondrial matrix, peroxisome, cytosolOne of 5 routes