4-hydroxy-2-oxoglutaric acid

Three steps

• heptadecanoyl-CoAReach from here →
  1. 4-hydroxy-2-oxoglutarate glyoxylate-lyase (pyruvate-forming)
  2. acetyl-CoA[m] + glyoxalate[m] + H2O[m] = CoA[m] + H+[m] + malate[m]
  3. long-chain-fatty-acid-CoA ligase (margaric acid)

  Catalysed by HOGA1, CLYBL, SLC27A2, ACSL4 and 6 moremitochondrial matrix, cytosolOne of 6 routes

• (9Z)-heptadecenoyl-CoAReach from here →
  1. 4-hydroxy-2-oxoglutarate glyoxylate-lyase (pyruvate-forming)
  2. acetyl-CoA[m] + glyoxalate[m] + H2O[m] = CoA[m] + H+[m] + malate[m]
  3. long-chain-fatty-acid-CoA ligase (9-heptadecylenic acid)

  Catalysed by HOGA1, CLYBL, SLC27A2, ACSL4 and 6 moremitochondrial matrix, cytosolOne of 5 routes

• (11Z,14Z,17Z)-eicosatrienoyl-CoAReach from here →
  1. 4-hydroxy-2-oxoglutarate glyoxylate-lyase (pyruvate-forming)
  2. acetyl-CoA[m] + glyoxalate[m] + H2O[m] = CoA[m] + H+[m] + malate[m]
  3. long-chain-fatty-acid-CoA ligase ((11Z,14Z,17Z)-eicosatrienoic acid)

  Catalysed by HOGA1, CLYBL, SLC27A2, ACSL4 and 6 moremitochondrial matrix, cytosolOne of 5 routes

• (2S)-2-aminobutanoateReach from here →
  1. 4-hydroxy-2-oxoglutarate glyoxylate-lyase (pyruvate-forming)
  2. L-Alanine:glyoxylate aminotransferase
  3. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine

  Catalysed by HOGA1, AGXT, AGXT2mitochondrial matrixOne of 7 routes

• nonadecanoyl-CoAReach from here →
  1. 4-hydroxy-2-oxoglutarate glyoxylate-lyase (pyruvate-forming)
  2. acetyl-CoA[m] + glyoxalate[m] + H2O[m] = CoA[m] + H+[m] + malate[m]
  3. long-chain-fatty-acid-CoA ligase (nonadecylic acid)

  Catalysed by HOGA1, CLYBL, SLC27A2, ACSL4 and 6 moremitochondrial matrix, cytosolOne of 6 routes

• (11Z)-octadecenoyl-CoAReach from here →
  1. 4-hydroxy-2-oxoglutarate glyoxylate-lyase (pyruvate-forming)
  2. acetyl-CoA[m] + glyoxalate[m] + H2O[m] = CoA[m] + H+[m] + malate[m]
  3. Transport of 11-Octadecenoyl Carnitine into the Mitochondrial Matrix

  Catalysed by HOGA1, CLYBL, CPT1A, CPT1C and 1 moremitochondrial matrix, cytosolOne of 3 routes

• dodecanedioyl-CoAReach from here →
  1. 4-hydroxy-2-oxoglutarate glyoxylate-lyase (pyruvate-forming)
  2. acetyl-CoA[m] + glyoxalate[m] + H2O[m] = CoA[m] + H+[m] + malate[m]
  3. Activation of dodecanedioic acid

  Catalysed by HOGA1, CLYBLmitochondrial matrix, cytosolOne of 4 routes

• decanedioyl-CoAReach from here →
  1. 4-hydroxy-2-oxoglutarate glyoxylate-lyase (pyruvate-forming)
  2. acetyl-CoA[m] + glyoxalate[m] + H2O[m] = CoA[m] + H+[m] + malate[m]
  3. Production of Sebacoylcarnitine

  Catalysed by HOGA1, CLYBL, CPT1A, CPT1C and 1 moremitochondrial matrix, cytosolOne of 4 routes

• octanedioyl-CoAReach from here →
  1. 4-hydroxy-2-oxoglutarate glyoxylate-lyase (pyruvate-forming)
  2. acetyl-CoA[m] + glyoxalate[m] + H2O[m] = CoA[m] + H+[m] + malate[m]
  3. Production of Suberylcarnitine

  Catalysed by HOGA1, CLYBL, CPT1A, CPT1C and 1 moremitochondrial matrix, cytosolOne of 4 routes

• hexanedioyl-CoAReach from here →
  1. 4-hydroxy-2-oxoglutarate glyoxylate-lyase (pyruvate-forming)
  2. acetyl-CoA[m] + glyoxalate[m] + H2O[m] = CoA[m] + H+[m] + malate[m]
  3. Activation of adipic acid for Formation of Adipoyl Carnitine

  Catalysed by HOGA1, CLYBLmitochondrial matrix, cytosolOne of 4 routes

• (4Z,7Z,10Z,13Z,16Z)-docosapentaenoyl-CoAReach from here →
  1. 4-hydroxy-2-oxoglutarate glyoxylate-lyase (pyruvate-forming)
  2. acetyl-CoA[m] + glyoxalate[m] + H2O[m] = CoA[m] + H+[m] + malate[m]
  3. long-chain-fatty-acid-CoA ligase ((4Z,7Z,10Z,13Z,16Z)-DPA)

  Catalysed by HOGA1, CLYBL, SLC27A2, ACSL4 and 6 moremitochondrial matrix, cytosolOne of 7 routes

• (11Z,14Z)-eicosadienoyl-CoAReach from here →
  1. 4-hydroxy-2-oxoglutarate glyoxylate-lyase (pyruvate-forming)
  2. acetyl-CoA[m] + glyoxalate[m] + H2O[m] = CoA[m] + H+[m] + malate[m]
  3. long-chain-fatty-acid-CoA ligase ((11Z,14Z)-eicosadienoic acid)

  Catalysed by HOGA1, CLYBL, SLC27A2, ACSL4 and 6 moremitochondrial matrix, cytosolOne of 5 routes

• 2-iminobutanoateReach from here →
  1. 4-hydroxy-2-oxoglutarate glyoxylate-lyase (pyruvate-forming)
  2. 2-oxobutanoate + L-alanine = (2S)-2-aminobutanoate + pyruvate
  3. 2-iminobutanoate + H2O = 2-oxobutanoate + NH4(+)

  Catalysed by HOGA1, AGXT2, RIDAmitochondrial matrix

• (R)-3-hydroxy-(11Z)-octadecenoyl-CoAReach from here →
  1. 4-hydroxy-2-oxoglutarate glyoxylate-lyase (pyruvate-forming)
  2. acetyl-CoA[m] + glyoxalate[m] + H2O[m] = CoA[m] + H+[m] + malate[m]
  3. Production of 3-Hydroxyoctadecenoylcarnitinec

  Catalysed by HOGA1, CLYBL, CPT1A, CPT1C and 1 moremitochondrial matrix, cytosol

• (13Z)-eicosenoyl-CoAReach from here →
  1. 4-hydroxy-2-oxoglutarate glyoxylate-lyase (pyruvate-forming)
  2. acetyl-CoA[m] + glyoxalate[m] + H2O[m] = CoA[m] + H+[m] + malate[m]
  3. long-chain-fatty-acid-CoA ligase ((13Z)-eicosenoic acid)

  Catalysed by HOGA1, CLYBL, SLC27A2, ACSL4 and 6 moremitochondrial matrix, cytosolOne of 5 routes

• 2-aminoprop-2-enoateReach from here →
  1. 4-hydroxy-2-oxoglutarate glyoxylate-lyase (pyruvate-forming)
  2. D-serine = pyruvate + NH4(+)
  3. D-serine = 2-aminoprop-2-enoate + H2O

  Catalysed by HOGA1, SRRmitochondrial matrix

• 4,8-dimethylnonanoyl-CoAReach from here →
  1. 4-hydroxy-2-oxoglutarate glyoxylate-lyase (pyruvate-forming)
  2. acetyl-CoA[m] + glyoxalate[m] + H2O[m] = CoA[m] + H+[m] + malate[m]
  3. Carnitine Dimethyl Nonanoyl Transferase, Revsible, Peroxisomal

  Catalysed by HOGA1, CLYBL, CRAT, CROTmitochondrial matrix, peroxisomeOne of 3 routes

• tetradecanedioyl-CoAReach from here →
  1. 4-hydroxy-2-oxoglutarate glyoxylate-lyase (pyruvate-forming)
  2. acetyl-CoA[m] + glyoxalate[m] + H2O[m] = CoA[m] + H+[m] + malate[m]
  3. Fatty Acid Beta Oxidation (C16Dc->C14Dc), Peroxisomal

  Catalysed by HOGA1, CLYBL, ACAA1, HSD17B4 and 2 moremitochondrial matrix, peroxisome

• hexadecanedioyl-CoAReach from here →
  1. 4-hydroxy-2-oxoglutarate glyoxylate-lyase (pyruvate-forming)
  2. acetyl-CoA[m] + glyoxalate[m] + H2O[m] = CoA[m] + H+[m] + malate[m]
  3. Production of Hexadecanedioylcarnitine

  Catalysed by HOGA1, CLYBL, CPT1A, CPT1C and 1 moremitochondrial matrix, cytosolOne of 2 routes

• pristanoyl-CoAReach from here →
  1. 4-hydroxy-2-oxoglutarate glyoxylate-lyase (pyruvate-forming)
  2. acetyl-CoA[m] + glyoxalate[m] + H2O[m] = CoA[m] + H+[m] + malate[m]
  3. fatty-acid-CoA ligase

  Catalysed by HOGA1, CLYBL, SLC27A2mitochondrial matrix, cytosolOne of 2 routes

• undecanoyl-CoAReach from here →
  1. 4-hydroxy-2-oxoglutarate glyoxylate-lyase (pyruvate-forming)
  2. acetyl-CoA[m] + glyoxalate[m] + H2O[m] = CoA[m] + H+[m] + malate[m]
  3. medium-chain acyl-CoA ligase (undecylic acid)

  Catalysed by HOGA1, CLYBL, ACSM4, ACSM1 and 4 moremitochondrial matrix, cytosolOne of 3 routes

• glycodeoxycholateReach from here →
  1. 4-hydroxy-2-oxoglutarate glyoxylate-lyase (pyruvate-forming)
  2. L-Alanine:glyoxylate aminotransferase
  3. Glycodeoxycholate amidohydrolase

  Catalysed by HOGA1, AGXT, AGXT2mitochondrial matrix, cytosolOne of 2 routes

• (5Z)-tetradecenoyl-CoAReach from here →
  1. 4-hydroxy-2-oxoglutarate glyoxylate-lyase (pyruvate-forming)
  2. acetyl-CoA[m] + glyoxalate[m] + H2O[m] = CoA[m] + H+[m] + malate[m]
  3. long-chain-fatty-acid-CoA ligase (physeteric acid)

  Catalysed by HOGA1, CLYBL, SLC27A2, ACSL4 and 6 moremitochondrial matrix, cytosolOne of 8 routes

• (3E)-decenoyl-CoAReach from here →
  1. 4-hydroxy-2-oxoglutarate glyoxylate-lyase (pyruvate-forming)
  2. acetyl-CoA[m] + glyoxalate[m] + H2O[m] = CoA[m] + H+[m] + malate[m]
  3. Fatty Acid Beta Oxidation (C12:1->C10:1), Mitochondrial

  Catalysed by HOGA1, CLYBL, ACADM, ECHS1 and 2 moremitochondrial matrix

• N-tetradecanoylglycineReach from here →
  1. 4-hydroxy-2-oxoglutarate glyoxylate-lyase (pyruvate-forming)
  2. L-Alanine:glyoxylate aminotransferase
  3. tetradecanoyl-CoA + glycine = N-tetradecanoylglycine + CoA + H(+)

  Catalysed by HOGA1, AGXT, AGXT2, GLYATL2mitochondrial matrix

• (2R,6)-dimethylheptanoyl-CoAReach from here →
  1. 4-hydroxy-2-oxoglutarate glyoxylate-lyase (pyruvate-forming)
  2. acetyl-CoA[m] + glyoxalate[m] + H2O[m] = CoA[m] + H+[m] + malate[m]
  3. acetyl-CoA C-acyltransferase (3-oxo-4(R),8-dimethyl-nonanoyl-CoA)

  Catalysed by HOGA1, CLYBL, HADHA, ACAA2 and 1 moremitochondrial matrix

• N-eicosanoylglycinateReach from here →
  1. 4-hydroxy-2-oxoglutarate glyoxylate-lyase (pyruvate-forming)
  2. L-Alanine:glyoxylate aminotransferase
  3. eicosanoyl-CoA + glycine = N-eicosanoylglycinate + CoA + H(+)

  Catalysed by HOGA1, AGXT, AGXT2, BAATmitochondrial matrix

• N-docosanoylglycineReach from here →
  1. 4-hydroxy-2-oxoglutarate glyoxylate-lyase (pyruvate-forming)
  2. L-Alanine:glyoxylate aminotransferase
  3. docosanoyl-CoA + glycine = N-docosanoylglycine + CoA + H(+)

  Catalysed by HOGA1, AGXT, AGXT2, BAATmitochondrial matrix

• N-hexacosanoylglycineReach from here →
  1. 4-hydroxy-2-oxoglutarate glyoxylate-lyase (pyruvate-forming)
  2. L-Alanine:glyoxylate aminotransferase
  3. hexacosanoyl-CoA + glycine = N-hexacosanoylglycine + CoA + H(+)

  Catalysed by HOGA1, AGXT, AGXT2, BAATmitochondrial matrix

• (7Z)-hexadecenoyl-CoAReach from here →
  1. 4-hydroxy-2-oxoglutarate glyoxylate-lyase (pyruvate-forming)
  2. acetyl-CoA[m] + glyoxalate[m] + H2O[m] = CoA[m] + H+[m] + malate[m]
  3. long-chain-fatty-acid-CoA ligase (7-palmitoleic acid)

  Catalysed by HOGA1, CLYBL, SLC27A2, ACSL4 and 6 moremitochondrial matrix, cytosolOne of 8 routes

• tricosanoyl-CoAReach from here →
  1. 4-hydroxy-2-oxoglutarate glyoxylate-lyase (pyruvate-forming)
  2. acetyl-CoA[m] + glyoxalate[m] + H2O[m] = CoA[m] + H+[m] + malate[m]
  3. long-chain-fatty-acid-CoA ligase (tricosanoic acid)

  Catalysed by HOGA1, CLYBL, SLC27A2, ACSL4 and 6 moremitochondrial matrix, cytosolOne of 4 routes

• thromboxane B2Reach from here →
  1. 4-hydroxy-2-oxoglutarate glyoxylate-lyase (pyruvate-forming)
  2. (S)-lactate + NAD(+) = pyruvate + NADH + H(+)
  3. thromboxane B2(out) + 2 (S)-lactate(in) = thromboxane B2(in) + 2 (S)-lactate(out)

  Catalysed by HOGA1, LDHA, LDHB, LDHC and 3 moremitochondrial matrix, inner side of the membrane, outer side of the membraneOne of 2 routes

• thiamine(1+) diphosphateReach from here →
  1. 4-hydroxy-2-oxoglutarate glyoxylate-lyase (pyruvate-forming)
  2. pyruvate:thiamin diphosphate acetaldehydetransferase (decarboxylating)
  3. 2-(alpha-hydroxyethyl)thiamine-diphosphate[m] + lipoamide[m] = S-acetyldihydrolipoamide[m] + thiamin-PP[m]

  Catalysed by HOGA1, PDHA1, PDHB, PDHA2 and 1 moremitochondrial matrix