glyoxylic acid

Three steps

• butanoateReach from here →
  1. acetyl-CoA[m] + glyoxalate[m] + H2O[m] = CoA[m] + H+[m] + malate[m]
  2. Butanoate:CoA ligase (AMP-forming)
  3. butanoyl-CoA + H2O = butanoate + CoA + H(+)

  Catalysed by CLYBL, ACSM4, ACSM1, ACSM2A and 9 moremitochondrial matrix, cytosol

• 3-phenylpyruvateReach from here →
  1. L-Alanine:glyoxylate aminotransferase
  2. transport of glycine and phenylalanine (cytosol to extracellular)
  3. Phenylalanine Transaminase, Mitochondrial

  Catalysed by AGXT, AGXT2, SLC7A5, GOT2 and 1 moremitochondrial matrix, extracellular space, cytosolOne of 7 routes

• thiocyanateReach from here →
  1. L-Alanine:glyoxylate aminotransferase
  2. 2-oxo-3-sulfanylpropanoate + [thioredoxin]-dithiol = [thioredoxin]-disulfide + hydrogen sulfide + pyruvate + H(+)
  3. 3-mercaptopyruvate sulfurtransferase (hydrogen-cyanide)

  Catalysed by AGXT, AGXT2, MPST, TSTmitochondrial matrix, cytosolOne of 3 routes

• 4-fumarylacetoacetateReach from here →
  1. L-Alanine:glyoxylate aminotransferase
  2. transport of acetoacetate and pyruvate (cytosol to mitochondria)
  3. 4-fumarylacetoacetate + H2O = acetoacetate + fumarate + H(+)

  Catalysed by AGXT, AGXT2, FAHmitochondrial matrix, cytosolOne of 2 routes

• dTTPReach from here →
  1. L-Alanine:glyoxylate aminotransferase
  2. pyruvate kinase (dTDP)
  3. DTTP Nucleotidohydrolase

  Catalysed by AGXT, AGXT2, PKM, PKLR and 3 moremitochondrial matrix, cytosol, extracellular spaceOne of 11 routes

• spermine monoaldehydeReach from here →
  1. acetyl-CoA[m] + glyoxalate[m] + H2O[m] = CoA[m] + H+[m] + malate[m]
  2. diamine N-acetyltransferase (N1-acetylspermine)
  3. primary-amine oxidase (spermine)

  Catalysed by CLYBL, SAT1, SAT2, AOC2 and 2 moremitochondrial matrix, cytosolOne of 3 routes

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

  Catalysed by AGXT, AGXT2, SLC6A9, SLC6A14 and 2 moremitochondrial matrix, extracellular space, cytosolOne of 5 routes

• O-octanoyl-(R)-carnitineReach from here →
  1. acetyl-CoA[m] + glyoxalate[m] + H2O[m] = CoA[m] + H+[m] + malate[m]
  2. Carnitine O-Palmitoyltransferase
  3. solute carrier (L-carnitine)

  Catalysed by CLYBL, CPT1A, CPT1C, CPT1B and 1 moremitochondrial matrix, cytosolOne of 11 routes

• CHEBI:18132Reach from here →
  1. acetyl-CoA[m] + glyoxalate[m] + H2O[m] = CoA[m] + H+[m] + malate[m]
  2. Acetyl-CoA:choline O-acetyltransferase
  3. ATP:choline phosphotransferase

  Catalysed by CLYBL, CHAT, CHKA, CHKBmitochondrial matrix, nucleus, cytosol

• N-carbamoyl-beta-alanineReach from here →
  1. L-Alanine:glyoxylate aminotransferase
  2. transport of chloride and glycine (cytosol to extracellular)
  3. Transport of 3-Ureidopropionate

  Catalysed by AGXT, AGXT2, SLC6A9, SLC6A14mitochondrial matrix, extracellular space, cytosolOne of 2 routes

• dodecanoateReach from here →
  1. acetyl-CoA[m] + glyoxalate[m] + H2O[m] = CoA[m] + H+[m] + malate[m]
  2. long-chain-fatty-acid-CoA ligase (lauric acid)
  3. Lauroyl-CoA hydrolase

  Catalysed by CLYBL, SLC27A2, ACSL4, ACSL3 and 9 moremitochondrial matrix, cytosolOne of 2 routes

• N(2)-formyl-N(1)-(5-phospho-D-ribosyl)glycinamideReach from here →
  1. L-Alanine:glyoxylate aminotransferase
  2. 5-Phospho-D-ribosylamine:glycine ligase (ADP-forming)
  3. 10-formyltetrahydrofolate:5'-phosphoribosylglycinamide formyltransferase

  Catalysed by AGXT, AGXT2, GARTmitochondrial matrix, cytosol

• arsenic triglutathioneReach from here →
  1. L-Alanine:glyoxylate aminotransferase
  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 AGXT, AGXT2, GSS, AS3MTmitochondrial matrixOne of 3 routes

• N-(4-aminobutyl)-3-aminopropanalReach from here →
  1. acetyl-CoA[m] + glyoxalate[m] + H2O[m] = CoA[m] + H+[m] + malate[m]
  2. diamine N-acetyltransferase (N1-acetylspermidine)
  3. primary-amine oxidase (spermidine)

  Catalysed by CLYBL, SAT1, SAT2, AOC2 and 2 moremitochondrial matrix, cytosolOne of 3 routes

• 2-phenylethylamineReach from here →
  1. L-Alanine:glyoxylate aminotransferase
  2. transport of glycine and phenylalanine (cytosol to extracellular)
  3. L-phenylalanine carboxy-lyase (phenylethylamine-forming)

  Catalysed by AGXT, AGXT2, SLC7A5, DDCmitochondrial matrix, extracellular space, cytosol

• hydrogen cyanideReach from here →
  1. acetyl-CoA[m] + glyoxalate[m] + H2O[m] = CoA[m] + H+[m] + malate[m]
  2. transport of malate and sulfite (cytosol to mitochondria)
  3. thiosulfate:cyanide sulfurtranserase

  Catalysed by CLYBL, SLC25A10, MPST, TSTmitochondrial matrix, cytosolOne of 2 routes

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

  Catalysed by AGXT, AGXT2, SLC7A5, PFASmitochondrial matrix, extracellular space, cytosol

• (2E,5Z,8Z,11Z,14Z,17Z)-eicosahexaenoyl-CoAReach from here →
  1. acetyl-CoA[m] + glyoxalate[m] + H2O[m] = CoA[m] + H+[m] + malate[m]
  2. long-chain-fatty-acid-CoA ligase (EPA)
  3. (5Z,8Z,11Z,14Z,17Z)-eicosapentaenoyl-CoA + O2 = (2E,5Z,8Z,11Z,14Z,17Z)-eicosahexaenoyl-CoA + H2O2

  Catalysed by CLYBL, SLC27A2, ACSL4, ACSL3 and 6 moremitochondrial matrix, cytosol

• gamma-L-glutamyl-(2S)-2-aminobutanoateReach from here →
  1. L-Alanine:glyoxylate aminotransferase
  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 AGXT, AGXT2, GCLCmitochondrial matrix

• 1-octadecanoyl-2-acyl-sn-glycero-3-phosphoethanolamineReach from here →
  1. acetyl-CoA[m] + glyoxalate[m] + H2O[m] = CoA[m] + H+[m] + malate[m]
  2. long-chain-fatty-acid-CoA ligase (stearate)
  3. a 2-acyl-sn-glycero-3-phosphoethanolamine + octadecanoyl-CoA = 1-octadecanoyl-2-acyl-sn-glycero-3-phosphoethanolamine + CoA

  Catalysed by CLYBL, SLC27A2, ACSL4, ACSL3 and 5 moremitochondrial matrix, cytosol

• S-methylmercury-L-cysteineReach from here →
  1. L-Alanine:glyoxylate aminotransferase
  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 AGXT, AGXT2, SLC7A5, SLC7A8mitochondrial matrix, extracellular space, cytosol, inner side of the membrane, outer side of the membraneOne of 6 routes

• 2-(alpha-Hydroxypropyl)thiamine diphosphateReach from here →
  1. L-Alanine:glyoxylate aminotransferase
  2. 2-oxobutanoate + L-alanine = (2S)-2-aminobutanoate + pyruvate
  3. branched chain keto acid dehydrogenase E1 (2-oxobutyrate)

  Catalysed by AGXT, AGXT2, BCKDHA, BCKDHBmitochondrial matrix

• 2-aminoisobutanoateReach from here →
  1. L-Alanine:glyoxylate aminotransferase
  2. transport of alanine and glycine (cytosol to extracellular)
  3. 2-aminoisobutanoate(out) + L-alanine(in) = 2-aminoisobutanoate(in) + L-alanine(out)

  Catalysed by AGXT, AGXT2, SLC3A2, SLC7A5 and 1 moremitochondrial matrix, extracellular space, cytosol, inner side of the membrane, outer side of the membraneOne of 2 routes

• D-isoleucineReach from here →
  1. L-Alanine:glyoxylate aminotransferase
  2. D-serine = pyruvate + NH4(+)
  3. D-isoleucine(out) + D-serine(in) = D-isoleucine(in) + D-serine(out)

  Catalysed by AGXT, AGXT2, SRR, SLC7A10mitochondrial matrix, inner side of the membrane, outer side of the membrane

• 15-deoxy-Delta(12,14)-prostaglandin J2-S-(R)-glutathioneReach from here →
  1. L-Alanine:glyoxylate aminotransferase
  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 AGXT, AGXT2, GSS, MGST3mitochondrial matrix

• 11-cis-retinyl esterReach from here →
  1. acetyl-CoA[m] + glyoxalate[m] + H2O[m] = CoA[m] + H+[m] + malate[m]
  2. Palmitoyl-CoA:retinol O-acyltransferase
  3. 11-cis-retinol + a fatty acyl-CoA = 11-cis-retinyl ester + CoA

  Catalysed by CLYBL, DGAT1, LRAT, AWAT1 and 2 moremitochondrial matrix, cytosol

• (6R)-10-formyltetrahydrofolateReach from here →
  1. L-Alanine:glyoxylate aminotransferase
  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 AGXT, AGXT2, SHMT1, SHMT2 and 2 moremitochondrial matrix

• (5S)-hydroxy-(6E,8Z,11Z,14Z,17Z)-eicosapentaenoateReach from here →
  1. L-Alanine:glyoxylate aminotransferase
  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 AGXT, AGXT2, GSS, GPX1 and 1 moremitochondrial matrix

• (12S)-hydroxy-(5Z,8Z,10E,14Z,17Z)-eicosapentaenoateReach from here →
  1. L-Alanine:glyoxylate aminotransferase
  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 AGXT, AGXT2, GSS, GPX1 and 1 moremitochondrial matrix

• (15S)-hydroxy-(11Z,13E)-eicosadienoateReach from here →
  1. L-Alanine:glyoxylate aminotransferase
  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 AGXT, AGXT2, GSS, GPX1 and 1 moremitochondrial matrix

• (17S)-hydroxy-(4Z,7Z,10Z,13Z,15E,19Z)-docosahexaenoateReach from here →
  1. L-Alanine:glyoxylate aminotransferase
  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 AGXT, AGXT2, GSS, GPX1 and 1 moremitochondrial matrix

• a hydroxy-1,2-diacyl-glycero-3-phosphocholineReach from here →
  1. L-Alanine:glyoxylate aminotransferase
  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 AGXT, AGXT2, GSS, GPX4mitochondrial matrix

• glycodeoxycholate 3-sulfateReach from here →
  1. L-Alanine:glyoxylate aminotransferase
  2. Glycodeoxycholate amidohydrolase
  3. glycodeoxycholate + 3'-phosphoadenylyl sulfate = glycodeoxycholate 3-sulfate + adenosine 3',5'-bisphosphate + H(+)

  Catalysed by AGXT, AGXT2, SULT2A1mitochondrial matrix, cytosol

• 1-butanoyl-2-(9Z-octadecenoyl)-glycerolReach from here →
  1. acetyl-CoA[m] + glyoxalate[m] + H2O[m] = CoA[m] + H+[m] + malate[m]
  2. Butanoate:CoA ligase (AMP-forming)
  3. 2-(9Z-octadecenoyl)-glycerol + butanoyl-CoA = 1-butanoyl-2-(9Z-octadecenoyl)-glycerol + CoA

  Catalysed by CLYBL, ACSM4, ACSM1, ACSM2A and 5 moremitochondrial matrix, cytosol

• 1-octanoyl-2-(9Z-octadecenoyl)-glycerolReach from here →
  1. acetyl-CoA[m] + glyoxalate[m] + H2O[m] = CoA[m] + H+[m] + malate[m]
  2. Octanoyl-CoA:L-carnitine O-octanoyltransferase
  3. 2-(9Z-octadecenoyl)-glycerol + octanoyl-CoA = 1-octanoyl-2-(9Z-octadecenoyl)-glycerol + CoA

  Catalysed by CLYBL, CROT, MOGAT2, MOGAT1mitochondrial matrix

• 1-eicosanoyl-2-(9Z-octadecenoyl)-glycerolReach from here →
  1. acetyl-CoA[m] + glyoxalate[m] + H2O[m] = CoA[m] + H+[m] + malate[m]
  2. long-chain-fatty-acid-CoA ligase (eicosanoate)
  3. eicosanoyl-CoA + 2-(9Z-octadecenoyl)-glycerol = 1-eicosanoyl-2-(9Z-octadecenoyl)-glycerol + CoA

  Catalysed by CLYBL, SLC27A2, ACSL4, ACSL3 and 7 moremitochondrial matrix, cytosol

• (6R)-5,10-methylenetetrahydrofolic acidReach from here →
  1. L-Alanine:glyoxylate aminotransferase
  2. 5,10-Methylenetetrahydrofolate:glycine hydroxymethyltransferase
  3. 5,10-Methylenetetrahydrofolate:glycine hydroxymethyltransferase

  Catalysed by AGXT, AGXT2, SHMT1mitochondrial matrix, cytosolOne of 7 routes

• O-butanoyl-(R)-carnitineReach from here →
  1. acetyl-CoA[m] + glyoxalate[m] + H2O[m] = CoA[m] + H+[m] + malate[m]
  2. Butanoate:CoA ligase (AMP-forming)
  3. butanoyl-CoA + (R)-carnitine = O-butanoyl-(R)-carnitine + CoA

  Catalysed by CLYBL, ACSM4, ACSM1, ACSM2A and 4 moremitochondrial matrix, cytosol

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

  Catalysed by AGXT, AGXT2, SLC3A2, SLC7A5 and 2 moremitochondrial matrix, extracellular space, cytosol

• (S)-lactoyl-AMPReach from here →
  1. L-Alanine:glyoxylate aminotransferase
  2. (S)-lactate + NAD(+) = pyruvate + NADH + H(+)
  3. (S)-lactate + ATP + H(+) = (S)-lactoyl-AMP + diphosphate

  Catalysed by AGXT, AGXT2, LDHA, LDHB and 5 moremitochondrial matrix

• (S)-lactoyl-CoAReach from here →
  1. L-Alanine:glyoxylate aminotransferase
  2. (S)-lactate + NAD(+) = pyruvate + NADH + H(+)
  3. (S)-lactate + ATP + CoA = (S)-lactoyl-CoA + AMP + diphosphate

  Catalysed by AGXT, AGXT2, LDHA, LDHB and 4 moremitochondrial matrix

• N-acetylneuraminate 9-phosphateReach from here →
  1. L-Alanine:glyoxylate aminotransferase
  2. pyruvate kinase (dTDP)
  3. N-acetylneuraminate synthase

  Catalysed by AGXT, AGXT2, PKM, PKLR and 1 moremitochondrial matrix, cytosol

• 3-oxo-(9Z)-eicosenoyl-CoAReach from here →
  1. acetyl-CoA[m] + glyoxalate[m] + H2O[m] = CoA[m] + H+[m] + malate[m]
  2. carnitine O-palmitoyltransferase ((7Z)-octadecenoyl-CoA)
  3. very-long-chain 3-oxoacyl-CoA synthase ((7Z)-octadecenoyl-CoA)

  Catalysed by CLYBL, CPT1A, CPT1C, CPT1B and 7 moremitochondrial matrix, cytosol

• (2E,6Z,9Z,12Z)-octadecatetraenoyl-CoAReach from here →
  1. acetyl-CoA[m] + glyoxalate[m] + H2O[m] = CoA[m] + H+[m] + malate[m]
  2. long-chain-fatty-acid-CoA ligase (gamma-linolenate)
  3. (6Z,9Z,12Z)-octadecatrienoyl-CoA + oxidized [electron-transfer flavoprotein] + H(+) = (2E,6Z,9Z,12Z)-octadecatetraenoyl-CoA + reduced [electron-transfer flavoprotein]

  Catalysed by CLYBL, SLC27A2, ACSL4, ACSL3 and 8 moremitochondrial matrix, cytosolOne of 2 routes

• (2E,7Z,10Z)-hexadecatrienoyl-CoAReach from here →
  1. acetyl-CoA[m] + glyoxalate[m] + H2O[m] = CoA[m] + H+[m] + malate[m]
  2. Fatty Acid Beta Oxidation Trans (C18:2->C16:2), Mitochondrial
  3. (7Z,10Z)-hexadecadienoyl-CoA + oxidized [electron-transfer flavoprotein] + H(+) = (2E,7Z,10Z)-hexadecatrienoyl-CoA + reduced [electron-transfer flavoprotein]

  Catalysed by CLYBL, HADHA, ACADVL, HADHB and 2 moremitochondrial matrix

• (2E,4Z,7Z,10Z,13Z)-hexadecapentaenoyl-CoAReach from here →
  1. acetyl-CoA[m] + glyoxalate[m] + H2O[m] = CoA[m] + H+[m] + malate[m]
  2. Fatty Acid Beta Oxidation (C18:4->C16:4), Mitochondrial
  3. Fatty Acid Beta Oxidation (C16:4->C16:5), Mitochondrial

  Catalysed by CLYBL, HADHA, ACADVL, HADHBmitochondrial matrixOne of 4 routes

• (2E,7Z,10Z,13Z)-hexadecatetraenoyl-CoAReach from here →
  1. acetyl-CoA[m] + glyoxalate[m] + H2O[m] = CoA[m] + H+[m] + malate[m]
  2. Fatty Acid Beta Oxidation (C18:3->C16:3), Mitochondrial
  3. (7Z,10Z,13Z)-hexadecatrienoyl-CoA + oxidized [electron-transfer flavoprotein] + H(+) = (2E,7Z,10Z,13Z)-hexadecatetraenoyl-CoA + reduced [electron-transfer flavoprotein]

  Catalysed by CLYBL, HADHA, ACADVL, HADHB and 2 moremitochondrial matrix

• (2E,4Z,7Z)-decatrienoyl-CoAReach from here →
  1. acetyl-CoA[m] + glyoxalate[m] + H2O[m] = CoA[m] + H+[m] + malate[m]
  2. Fatty Acid Beta Oxidation (C12:3->C10:2), Mitochondrial
  3. Fatty Acid Beta Oxidation (C10:2->C10:3), Mitochondrial

  Catalysed by CLYBL, ECHS1, ACAA2, HADH and 1 moremitochondrial matrixOne of 3 routes

• 3-(glutathion-S-yl)-4-hydroxynonanalReach from here →
  1. L-Alanine:glyoxylate aminotransferase
  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 AGXT, AGXT2, GSS, GSTP1mitochondrial matrix

• glycoursodeoxycholate 3-sulfateReach from here →
  1. L-Alanine:glyoxylate aminotransferase
  2. glycine to glycoursodeoxycholate conversion
  3. glycoursodeoxycholate + 3'-phosphoadenylyl sulfate = glycoursodeoxycholate 3-sulfate + adenosine 3',5'-bisphosphate + H(+)

  Catalysed by AGXT, AGXT2, SULT2A1mitochondrial matrix, cytosol