L-2-amino-3-oxobutanoic acid

Three steps

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

• 15-deoxy-Delta(12,14)-prostaglandin J2-S-(R)-glutathioneReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  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 GCAT, GSS, MGST3mitochondrial matrix

• (6R)-10-formyltetrahydrofolateReach 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. (6S)-5,6,7,8-tetrahydrofolate + formate + ATP = (6R)-10-formyltetrahydrofolate + ADP + phosphate

  Catalysed by GCAT, SHMT1, SHMT2, MTHFD1 and 1 moremitochondrial matrix

• (5S)-hydroxy-(6E,8Z,11Z,14Z,17Z)-eicosapentaenoateReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  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 GCAT, GSS, GPX1, GPX4mitochondrial matrix

• (12S)-hydroxy-(5Z,8Z,10E,14Z,17Z)-eicosapentaenoateReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  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 GCAT, GSS, GPX1, GPX4mitochondrial matrix

• (15S)-hydroxy-(11Z,13E)-eicosadienoateReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  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 GCAT, GSS, GPX1, GPX4mitochondrial matrix

• (17S)-hydroxy-(4Z,7Z,10Z,13Z,15E,19Z)-docosahexaenoateReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  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 GCAT, GSS, GPX1, GPX4mitochondrial matrix

• a hydroxy-1,2-diacyl-glycero-3-phosphocholineReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  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 GCAT, GSS, GPX4mitochondrial matrix

• glycodeoxycholate 3-sulfateReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. Glycodeoxycholate amidohydrolase
  3. glycodeoxycholate + 3'-phosphoadenylyl sulfate = glycodeoxycholate 3-sulfate + adenosine 3',5'-bisphosphate + H(+)

  Catalysed by GCAT, SULT2A1mitochondrial matrix, cytosol

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

  Catalysed by GCAT, AADAT, AGXT2mitochondrial matrixOne of 7 routes

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

  Catalysed by GCAT, AADAT, AGXT2mitochondrial matrixOne of 3 routes

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

  Catalysed by GCAT, AADAT, AGXT2mitochondrial matrixOne of 3 routes

• (6R)-5,10-methylenetetrahydrofolic acidReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. 5,10-Methylenetetrahydrofolate:glycine hydroxymethyltransferase
  3. 5,10-Methylenetetrahydrofolate:glycine hydroxymethyltransferase

  Catalysed by GCAT, SHMT1mitochondrial matrix, cytosolOne of 7 routes

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

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

• (5Z)-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-7-enoyl-CoA)

  Catalysed by GCAT, BAAT, HADHA, ACAA2 and 1 moremitochondrial matrix, peroxisomeOne of 2 routes

• (11Z)-docosenoyl-CoA(4-)Reach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. bile acid-CoA: amino acid N-acyltransferase
  3. long-chain-fatty-acid-CoA ligase (cis-cetoleic acid)

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

• (7Z)-tetradecenoyl-CoAReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. bile acid-CoA: amino acid N-acyltransferase
  3. long-chain-fatty-acid-CoA ligase ((7Z)-tetradecenoic acid)

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

• (4Z,7Z,10Z,13Z)-hexadecatetraenoyl-CoAReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. bile acid-CoA: amino acid N-acyltransferase
  3. Fatty Acid Beta Oxidation (C18:4->C16:4), Mitochondrial

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

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

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

• (4Z,7Z)-decadienoyl-CoAReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. bile acid-CoA: amino acid N-acyltransferase
  3. Fatty Acid Beta Oxidation (C12:3->C10:2), Mitochondrial

  Catalysed by GCAT, BAAT, ECHS1, ACAA2 and 1 moremitochondrial matrix, peroxisomeOne of 2 routes

• (5Z)-octenoyl-CoAReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. bile acid-CoA: amino acid N-acyltransferase
  3. Fatty Acid Beta Oxidation (C10:2->C8:1), Mitochondrial

  Catalysed by GCAT, BAAT, ECHS1, ACAA2 and 1 moremitochondrial matrix, peroxisomeOne of 2 routes

• 3-(glutathion-S-yl)-4-hydroxynonanalReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  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 GCAT, GSS, GSTP1mitochondrial matrix

• glycoursodeoxycholate 3-sulfateReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. glycine to glycoursodeoxycholate conversion
  3. glycoursodeoxycholate + 3'-phosphoadenylyl sulfate = glycoursodeoxycholate 3-sulfate + adenosine 3',5'-bisphosphate + H(+)

  Catalysed by GCAT, SULT2A1mitochondrial matrix, cytosol

• glycochenodeoxycholate 3-sulfateReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. chenodeoxycholoyl-CoA + glycine = glycochenodeoxycholate + CoA + H(+)
  3. glycochenodeoxycholate + 3'-phosphoadenylyl sulfate = glycochenodeoxycholate 3-sulfate + adenosine 3',5'-bisphosphate + H(+)

  Catalysed by GCAT, BAAT, SULT2A1mitochondrial matrix

• deoxycholateReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. Glycodeoxycholate amidohydrolase
  3. Glycodeoxycholate amidohydrolase

  Catalysed by GCATmitochondrial matrix, cytosol

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

  Catalysed by GCAT, GSS, SQORmitochondrial matrix

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

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

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

  Catalysed by GCAT, SLC6A9, SLC6A14, SLC12A2 and 1 moremitochondrial matrix, extracellular space, cytosolOne of 2 routes

• CHEBI:27540Reach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. bile acid-CoA: amino acid N-acyltransferase
  3. medium-chain acyl-CoA ligase (hexanoic acid)

  Catalysed by GCAT, BAAT, ACSM4, ACSM1 and 4 moremitochondrial matrix, peroxisome, cytosolOne of 6 routes

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

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

• 5-hydroxytryptophanReach 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 2 routes

• 5(S)-HETEReach 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 2 routes

• CHEBI:28701Reach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. bile acid-CoA: amino acid N-acyltransferase
  3. Propanoyl-CoA:acetyl-CoA C-acyltransferase

  Catalysed by GCAT, BAAT, ACAA1, SCP2 and 1 moremitochondrial matrix, peroxisomeOne of 7 routes

• pyridoxine 5'-phosphateReach 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

• N-acetyl-L-cysteineReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. transport of cysteine and glycine (cytosol to extracellular)
  3. N-acetylglutamate synthase (N-acetyl-L-cysteine)

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

• a dicarboxylateReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. Hippurate (Benzoylglycinate) Formation
  3. N-benzoylglycine(out) + a dicarboxylate(in) = N-benzoylglycine(in) + a dicarboxylate(out)

  Catalysed by GCAT, SLC22A6, SLC22A8mitochondrial matrix, inner side of the membrane, outer side of the membraneOne of 2 routes

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

  Catalysed by GCAT, GSS, CLIC1, GSTO1 and 1 moremitochondrial matrix

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

  Catalysed by GCAT, SLC3A2, SLC7A5, SLC7A8 and 1 moremitochondrial matrix, extracellular space, cytosol, inner side of the membrane, outer side of the membraneOne of 2 routes

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

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

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

  Catalysed by GCAT, SLC7A5, FARSB, FARSAmitochondrial matrix, extracellular space, cytosol

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

  Catalysed by GCAT, SLC7A5, WARS1mitochondrial matrix, extracellular space, cytosol

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

  Catalysed by GCAT, SLC7A5, IARS1mitochondrial matrix, extracellular space, cytosol

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

  Catalysed by GCAT, SLC7A5, QARS1mitochondrial matrix, extracellular space, cytosol

• isocyanateReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. transport of citrulline and glycine (cytosol to extracellular)
  3. L-citrulline + H(+) = isocyanate + L-ornithine

  Catalysed by GCAT, SLC7A9, SLC3A1, SLC7A6 and 1 moremitochondrial matrix, extracellular space, cytosol

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

  Catalysed by GCAT, NARS1mitochondrial matrix, extracellular space, cytosol

• cholateReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. gamma-L-glutamyl-L-cysteine:glycine ligase (ADP-forming)
  3. solute carrier organic anion transporter (cholate)

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

• glycolateReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. glycine:oxygen oxidoreductase (deaminating)
  3. glycolate:NADP+ oxidoreductase

  Catalysed by GCAT, DAO, GRHPRmitochondrial matrix, peroxisome, cytosolOne of 3 routes

• hydrogen sulfideReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. transport of cysteine and glycine (cytosol to extracellular)
  3. L-cysteine hydrogen-sulfide-lyase (deaminating

  Catalysed by GCAT, SLC3A2, SLC7A5, SLC7A8 and 1 moremitochondrial matrix, extracellular space, cytosolOne of 3 routes

• L-aspartateReach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. transport of alanine and glycine (cytosol to extracellular)
  3. oxaloacetate + L-alanine = L-aspartate + pyruvate

  Catalysed by GCAT, SLC3A2, SLC7A5, SLC7A10 and 1 moremitochondrial matrix, extracellular space, cytosolOne of 21 routes

• CHEBI:30249Reach from here →
  1. Acetyl-CoA:glycine C-acetyltransferase
  2. transport of glycine and tryptophan (cytosol to extracellular)
  3. L-tryptophan:oxygen 2,3-oxidoreductase (decyclizing)

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