(2S)-2-aminobutanoate

Three steps

• trans-urocanateReach from here →
  1. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  2. transport of glycine and histidine (cytosol to extracellular)
  3. L-histidine ammonia-lyase (urocanate-forming)

  Catalysed by AGXT2, SLC7A6, HALextracellular space, cytosol

• urateReach from here →
  1. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  2. gamma-L-glutamyl-L-cysteine + glycine + ATP = glutathione + ADP + phosphate + H(+)
  3. urate(out) + glutathione(in) = urate(in) + glutathione(out)

  Catalysed by AGXT2, GSS, SLC22A13inner side of the membrane, outer side of the membrane

• 5-hydroxy-L-tryptophanReach from here →
  1. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  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 AGXT2, SLC7A5, TPH1, TPH2extracellular space, cytosol

• methylarsonous acidReach from here →
  1. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  2. gamma-L-glutamyl-L-cysteine + glycine + ATP = glutathione + ADP + phosphate + H(+)
  3. methylarsonate + 2 glutathione + H(+) = methylarsonous acid + glutathione disulfide + H2O

  Catalysed by AGXT2, GSS, GSTO1, GSTO2

• 4-methyl-2-oxopentanoateReach from here →
  1. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  2. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  3. L-leucine + glyoxylate = 4-methyl-2-oxopentanoate + glycine

  Catalysed by AGXT2, AADATOne of 5 routes

• CHEBI:17927Reach from here →
  1. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  2. bile acid-CoA: amino acid N-acyltransferase
  3. N1,N8-diacetylspermidine to N1-acetylspermidine conversion

  Catalysed by AGXT2, BAATperoxisome, cytosolOne of 2 routes

• 3-oxopropanoic acidReach from here →
  1. 2-oxobutanoate + L-alanine = (2S)-2-aminobutanoate + pyruvate
  2. beta-alanine(out) + L-alanine(in) = beta-alanine(in) + L-alanine(out)
  3. beta-alanine:2-oxoglutarate aminotransferase

  Catalysed by AGXT2, SLC7A10, ABATinner side of the membrane, outer side of the membrane, mitochondrial matrix

• 3-phenylpyruvateReach from here →
  1. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  2. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  3. glyoxylate + L-phenylalanine = 3-phenylpyruvate + glycine

  Catalysed by AGXT2, AADATOne of 6 routes

• dTDPReach from here →
  1. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  2. 2-oxobutanoate + L-alanine = (2S)-2-aminobutanoate + pyruvate
  3. pyruvate kinase (dTDP)

  Catalysed by AGXT2, PKM, PKLRcytosol

• CHEBI:18183Reach from here →
  1. 2-oxobutanoate + L-alanine = (2S)-2-aminobutanoate + pyruvate
  2. gamma-glutamyltransferase (alanine)
  3. gamma-glutamylcyclotransferase ((5-L-glutamyl)-L-amino acid)

  Catalysed by AGXT2, GGT1, GGT5, GGTLC2 and 5 moreextracellular space, cytosol

• N-carbamoyl-beta-alanineReach from here →
  1. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  2. transport of chloride and glycine (cytosol to extracellular)
  3. Transport of 3-Ureidopropionate

  Catalysed by AGXT2, SLC6A9, SLC6A14extracellular space, cytosolOne of 2 routes

• N(2)-formyl-N(1)-(5-phospho-D-ribosyl)glycinamideReach from here →
  1. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  2. 5-Phospho-D-ribosylamine:glycine ligase (ADP-forming)
  3. 10-formyltetrahydrofolate:5'-phosphoribosylglycinamide formyltransferase

  Catalysed by AGXT2, GARTcytosol

• arsenic triglutathioneReach from here →
  1. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  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 AGXT2, GSS, AS3MTOne of 3 routes

• 2-phenylethylamineReach from here →
  1. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  2. transport of glycine and phenylalanine (cytosol to extracellular)
  3. L-phenylalanine carboxy-lyase (phenylethylamine-forming)

  Catalysed by AGXT2, SLC7A5, DDCextracellular space, cytosol

• CHEBI:18405Reach from here →
  1. 2-oxobutanoate + L-alanine = (2S)-2-aminobutanoate + pyruvate
  2. L-selenocysteine selenide-lyase (L-alanine-forming)
  3. pyridoxine 5'-phosphate:oxygen oxidoreductase

  Catalysed by AGXT2, SCLY, PNPOcytosolOne of 3 routes

• 2-formamido-N(1)-(5-phospho-D-ribosyl)acetamidineReach from here →
  1. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  2. transport of glutamine and glycine (cytosol to extracellular)
  3. 5'-Phosphoribosylformylglycinamide:L-glutamine amido-ligase (ADP-forming)

  Catalysed by AGXT2, SLC7A5, PFASextracellular space, cytosol

• S-methylmercury-L-cysteineReach from here →
  1. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  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 AGXT2, SLC7A5, SLC7A8extracellular space, cytosol, inner side of the membrane, outer side of the membraneOne of 6 routes

• D-isoleucineReach from here →
  1. 2-oxobutanoate + L-alanine = (2S)-2-aminobutanoate + pyruvate
  2. D-serine(out) + L-alanine(in) = D-serine(in) + L-alanine(out)
  3. D-isoleucine(out) + D-serine(in) = D-isoleucine(in) + D-serine(out)

  Catalysed by AGXT2, SLC7A10inner side of the membrane, outer side of the membrane

• 15-deoxy-Delta(12,14)-prostaglandin J2-S-(R)-glutathioneReach from here →
  1. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  2. gamma-L-glutamyl-L-cysteine + glycine + ATP = glutathione + ADP + phosphate + H(+)
  3. 15-deoxy-Delta(12,14)-prostaglandin J2 + glutathione = 15-deoxy-Delta(12,14)-prostaglandin J2-S-(R)-glutathione

  Catalysed by AGXT2, GSS, MGST3

• Xaa-L-Pro dipeptideReach from here →
  1. 2-oxobutanoate + L-alanine = (2S)-2-aminobutanoate + pyruvate
  2. L-proline(out) + L-alanine(in) + Na(+)(out) = L-proline(in) + L-alanine(out) + Na(+)(in)
  3. Xaa-L-Pro dipeptide + H2O = an L-alpha-amino acid + L-proline

  Catalysed by AGXT2, SLC1A4, PEPDinner side of the membrane, outer side of the membrane

• (6R)-10-formyltetrahydrofolateReach from here →
  1. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  2. (6R)-5,10-methylene-5,6,7,8-tetrahydrofolate + glycine + H2O = (6S)-5,6,7,8-tetrahydrofolate + L-serine
  3. (6S)-5,6,7,8-tetrahydrofolate + formate + ATP = (6R)-10-formyltetrahydrofolate + ADP + phosphate

  Catalysed by AGXT2, SHMT1, SHMT2, MTHFD1 and 1 more

• (5S)-hydroxy-(6E,8Z,11Z,14Z,17Z)-eicosapentaenoateReach from here →
  1. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  2. gamma-L-glutamyl-L-cysteine + glycine + ATP = glutathione + ADP + phosphate + H(+)
  3. (5S)-hydroperoxy-(6E,8Z,11Z,14Z,17Z)-eicosapentaenoate + 2 glutathione = (5S)-hydroxy-(6E,8Z,11Z,14Z,17Z)-eicosapentaenoate + glutathione disulfide + H2O

  Catalysed by AGXT2, GSS, GPX1, GPX4

• (12S)-hydroxy-(5Z,8Z,10E,14Z,17Z)-eicosapentaenoateReach from here →
  1. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  2. gamma-L-glutamyl-L-cysteine + glycine + ATP = glutathione + ADP + phosphate + H(+)
  3. (12S)-hydroperoxy-(5Z,8Z,10E,14Z,17Z)-eicosapentaenoate + 2 glutathione = (12S)-hydroxy-(5Z,8Z,10E,14Z,17Z)-eicosapentaenoate + glutathione disulfide + H2O

  Catalysed by AGXT2, GSS, GPX1, GPX4

• (15S)-hydroxy-(11Z,13E)-eicosadienoateReach from here →
  1. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  2. gamma-L-glutamyl-L-cysteine + glycine + ATP = glutathione + ADP + phosphate + H(+)
  3. (15S)-hydroperoxy-(11Z,13E)-eicosadienoate + 2 glutathione = (15S)-hydroxy-(11Z,13E)-eicosadienoate + glutathione disulfide + H2O

  Catalysed by AGXT2, GSS, GPX1, GPX4

• (17S)-hydroxy-(4Z,7Z,10Z,13Z,15E,19Z)-docosahexaenoateReach from here →
  1. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  2. gamma-L-glutamyl-L-cysteine + glycine + ATP = glutathione + ADP + phosphate + H(+)
  3. (17S)-hydroperoxy-(4Z,7Z,10Z,13Z,15E,19Z)-docosahexaenoate + 2 glutathione = (17S)-hydroxy-(4Z,7Z,10Z,13Z,15E,19Z)-docosahexaenoate + glutathione disulfide + H2O

  Catalysed by AGXT2, GSS, GPX1, GPX4

• a hydroxy-1,2-diacyl-glycero-3-phosphocholineReach from here →
  1. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  2. gamma-L-glutamyl-L-cysteine + glycine + ATP = glutathione + ADP + phosphate + H(+)
  3. a hydroperoxy-1,2-diacyl-glycero-3-phosphocholine + 2 glutathione = a hydroxy-1,2-diacyl-glycero-3-phosphocholine + glutathione disulfide + H2O

  Catalysed by AGXT2, GSS, GPX4

• biocytinReach from here →
  1. N(omega),N(omega)-dimethyl-L-arginine + 2-oxobutanoate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + (2S)-2-aminobutanoate
  2. N(omega),N(omega)-dimethyl-L-arginine(in) + L-lysine(out) = N(omega),N(omega)-dimethyl-L-arginine(out) + L-lysine(in)
  3. biocytin + H2O = biotin + L-lysine

  Catalysed by AGXT2, SLC25A2, BTDinner side of the membrane, outer side of the membrane

• glycodeoxycholate 3-sulfateReach from here →
  1. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  2. Glycodeoxycholate amidohydrolase
  3. glycodeoxycholate + 3'-phosphoadenylyl sulfate = glycodeoxycholate 3-sulfate + adenosine 3',5'-bisphosphate + H(+)

  Catalysed by AGXT2, SULT2A1cytosol

• 3',5'-diiodo-L-thyronineReach from here →
  1. 2-oxobutanoate + L-alanine = (2S)-2-aminobutanoate + pyruvate
  2. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  3. 3',5'-diiodo-L-thyronine + iodide + A + H(+) = 3,3',5'-triiodo-L-thyronine + AH2

  Catalysed by AGXT2, MOCOS, DIO3

• 5-(3,3'-dimethylguanidino)-2-oxopentanoateReach from here →
  1. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  2. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  3. N(omega),N('omega)-dimethyl-L-arginine + glyoxylate = 5-(3,3'-dimethylguanidino)-2-oxopentanoate + glycine

  Catalysed by AGXT2One of 4 routes

• 5-(3-methylguanidino)-2-oxopentanoateReach from here →
  1. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  2. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  3. N(omega)-methyl-L-arginine + glyoxylate = 5-(3-methylguanidino)-2-oxopentanoate + glycine

  Catalysed by AGXT2One of 4 routes

• (6R)-5,10-methylenetetrahydrofolic acidReach from here →
  1. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  2. 5,10-Methylenetetrahydrofolate:glycine hydroxymethyltransferase
  3. 5,10-Methylenetetrahydrofolate:glycine hydroxymethyltransferase

  Catalysed by AGXT2, SHMT1cytosolOne of 7 routes

• S-disulfanyl-L-cysteineReach from here →
  1. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  2. transport of cysteine and glycine (cytosol to extracellular)
  3. S-sulfanyl-L-cysteine + L-cysteine = S-disulfanyl-L-cysteine + L-alanine

  Catalysed by AGXT2, SLC3A2, SLC7A5, SLC7A8 and 1 moreextracellular space, cytosol

• (5Z)-dodecenoyl-CoAReach from here →
  1. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  2. bile acid-CoA: amino acid N-acyltransferase
  3. acetyl-CoA C-acyltransferase (3-oxomyrist-7-enoyl-CoA)

  Catalysed by AGXT2, BAAT, HADHA, ACAA2 and 1 moreperoxisome, mitochondrial matrixOne of 2 routes

• 3-iodothyronamineReach from here →
  1. 2-oxobutanoate + L-alanine = (2S)-2-aminobutanoate + pyruvate
  2. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  3. 3-iodothyronamine + iodide + A + H(+) = 3,5-diiodothyronamine + AH2

  Catalysed by AGXT2, MOCOS, DIO1, DIO3One of 2 routes

• (11Z)-docosenoyl-CoA(4-)Reach from here →
  1. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  2. bile acid-CoA: amino acid N-acyltransferase
  3. long-chain-fatty-acid-CoA ligase (cis-cetoleic acid)

  Catalysed by AGXT2, BAAT, SLC27A2, ACSL4 and 6 moreperoxisome, cytosolOne of 5 routes

• 3-iodo-L-thyronineReach from here →
  1. 2-oxobutanoate + L-alanine = (2S)-2-aminobutanoate + pyruvate
  2. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  3. 3-iodo-L-thyronine + iodide + A + H(+) = 3,5-diiodo-L-thyronine + AH2

  Catalysed by AGXT2, MOCOS, DIO1, DIO3One of 2 routes

• (7Z)-tetradecenoyl-CoAReach from here →
  1. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  2. bile acid-CoA: amino acid N-acyltransferase
  3. long-chain-fatty-acid-CoA ligase ((7Z)-tetradecenoic acid)

  Catalysed by AGXT2, BAAT, SLC27A2, ACSL4 and 6 moreperoxisome, cytosolOne of 8 routes

• 3'-iodo-L-thyronineReach from here →
  1. 2-oxobutanoate + L-alanine = (2S)-2-aminobutanoate + pyruvate
  2. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  3. 3'-iodo-L-thyronine + iodide + A + H(+) = 3',5'-diiodo-L-thyronine + AH2

  Catalysed by AGXT2, MOCOS, DIO1, DIO2One of 2 routes

• L-thyronineReach from here →
  1. 2-oxobutanoate + L-alanine = (2S)-2-aminobutanoate + pyruvate
  2. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  3. L-thyronine + iodide + A + H(+) = 3-iodo-L-thyronine + AH2

  Catalysed by AGXT2, MOCOS, DIO3

• thyronamineReach from here →
  1. 2-oxobutanoate + L-alanine = (2S)-2-aminobutanoate + pyruvate
  2. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  3. thyronamine + iodide + A + H(+) = 3-iodothyronamine + AH2

  Catalysed by AGXT2, MOCOS, DIO3

• 3'-iodothyronamineReach from here →
  1. 2-oxobutanoate + L-alanine = (2S)-2-aminobutanoate + pyruvate
  2. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  3. 3'-iodothyronamine + iodide + A + H(+) = 3',5'-diiodothyronamine + AH2

  Catalysed by AGXT2, MOCOS, DIO1, DIO2One of 2 routes

• 3,3'-diiodothyronamineReach from here →
  1. 2-oxobutanoate + L-alanine = (2S)-2-aminobutanoate + pyruvate
  2. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  3. 3,3'-diiodothyronamine + iodide + A + H(+) = 3,3',5'-triiodothyronamine + AH2

  Catalysed by AGXT2, MOCOS, DIO1, DIO2One of 2 routes

• 3',5'-diiodothyronamineReach from here →
  1. 2-oxobutanoate + L-alanine = (2S)-2-aminobutanoate + pyruvate
  2. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  3. 3',5'-diiodothyronamine + iodide + A + H(+) = 3,3',5'-triiodothyronamine + AH2

  Catalysed by AGXT2, MOCOS, DIO3

• 3,3',5'-triiodothyronamineReach from here →
  1. 2-oxobutanoate + L-alanine = (2S)-2-aminobutanoate + pyruvate
  2. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  3. 3,3',5'-triiodothyronamine + iodide + A + H(+) = 3,3',5,5'-tetraiodothyronamine + AH2

  Catalysed by AGXT2, MOCOS, DIO3

• (4Z,7Z,10Z,13Z)-hexadecatetraenoyl-CoAReach from here →
  1. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  2. bile acid-CoA: amino acid N-acyltransferase
  3. Fatty Acid Beta Oxidation (C18:4->C16:4), Mitochondrial

  Catalysed by AGXT2, BAAT, HADHA, ACADVL and 1 moreperoxisome, mitochondrial matrixOne of 3 routes

• (7Z,10Z)-hexadecadienoyl-CoAReach from here →
  1. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  2. bile acid-CoA: amino acid N-acyltransferase
  3. Fatty Acid Beta Oxidation Trans (C18:2->C16:2), Mitochondrial

  Catalysed by AGXT2, BAAT, HADHA, ACADVL and 1 moreperoxisome, mitochondrial matrix

• (4Z,7Z)-decadienoyl-CoAReach from here →
  1. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  2. bile acid-CoA: amino acid N-acyltransferase
  3. Fatty Acid Beta Oxidation (C12:3->C10:2), Mitochondrial

  Catalysed by AGXT2, BAAT, ECHS1, ACAA2 and 1 moreperoxisome, mitochondrial matrixOne of 2 routes

• (5Z)-octenoyl-CoAReach from here →
  1. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  2. bile acid-CoA: amino acid N-acyltransferase
  3. Fatty Acid Beta Oxidation (C10:2->C8:1), Mitochondrial

  Catalysed by AGXT2, BAAT, ECHS1, ACAA2 and 1 moreperoxisome, mitochondrial matrixOne of 2 routes

• 3-(glutathion-S-yl)-4-hydroxynonanalReach from here →
  1. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  2. gamma-L-glutamyl-L-cysteine + glycine + ATP = glutathione + ADP + phosphate + H(+)
  3. (E)-4-hydroxynon-2-enal + glutathione = 3-(glutathion-S-yl)-4-hydroxynonanal

  Catalysed by AGXT2, GSS, GSTP1