4-hydroxy-2-oxoglutaric acid

Three steps

• 3-hydroxy-2-methylpropanoateReach from here →
  1. 4-hydroxy-2-oxoglutarate glyoxylate-lyase (pyruvate-forming)
  2. (R)-3-amino-2-methylpropanoate + pyruvate = 2-methyl-3-oxopropanoate + L-alanine
  3. 3-hydroxy-2-methylpropanoate + NAD(+) = 2-methyl-3-oxopropanoate + NADH + H(+)

  Catalysed by HOGA1, AGXT2, HIBADHmitochondrial matrix

• glycoursodeoxycholateReach from here →
  1. 4-hydroxy-2-oxoglutarate glyoxylate-lyase (pyruvate-forming)
  2. L-Alanine:glyoxylate aminotransferase
  3. glycine to glycoursodeoxycholate conversion

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

• prostaglandin E3Reach from here →
  1. 4-hydroxy-2-oxoglutarate glyoxylate-lyase (pyruvate-forming)
  2. (S)-Lactate:NAD+ oxidoreductase
  3. Transport of Prostaglandin E3, Lactate Antiport

  Catalysed by HOGA1, LDHB, LDHAL6Bmitochondrial matrix, extracellular space, cytosolOne of 4 routes

• prostaglandin A2Reach from here →
  1. 4-hydroxy-2-oxoglutarate glyoxylate-lyase (pyruvate-forming)
  2. (S)-Lactate:NAD+ oxidoreductase
  3. Transport of Prostaglandin A2, Lactate Antiport

  Catalysed by HOGA1, LDHB, LDHAL6Bmitochondrial matrix, extracellular space, cytosolOne of 2 routes

• prostaglandin J2Reach from here →
  1. 4-hydroxy-2-oxoglutarate glyoxylate-lyase (pyruvate-forming)
  2. (S)-Lactate:NAD+ oxidoreductase
  3. Transport of Prostaglandin J2, Lactate Antiport

  Catalysed by HOGA1, LDHB, LDHAL6Bmitochondrial matrix, extracellular space, cytosolOne of 2 routes

• 15-oxoprostaglandin F2alphaReach from here →
  1. 4-hydroxy-2-oxoglutarate glyoxylate-lyase (pyruvate-forming)
  2. (S)-Lactate:NAD+ oxidoreductase
  3. Transport of 15-Keto-Prostaglandin F2A, Lactate Antiport

  Catalysed by HOGA1, LDHB, LDHAL6Bmitochondrial matrix, extracellular space, cytosolOne of 2 routes

• N-(9Z-octadecenoyl)glycineReach from here →
  1. 4-hydroxy-2-oxoglutarate glyoxylate-lyase (pyruvate-forming)
  2. L-Alanine:glyoxylate aminotransferase
  3. (9Z)-octadecenoyl-CoA + glycine = N-(9Z-octadecenoyl)glycine + CoA + H(+)

  Catalysed by HOGA1, AGXT, AGXT2, GLYATL3 and 1 moremitochondrial matrix

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

  Catalysed by HOGA1, AGXT, AGXT2, GLYATL2mitochondrial matrix

• N-(9Z-hexadecenoyl)-glycineReach from here →
  1. 4-hydroxy-2-oxoglutarate glyoxylate-lyase (pyruvate-forming)
  2. L-Alanine:glyoxylate aminotransferase
  3. (9Z)-hexadecenoyl-CoA + glycine = N-(9Z-hexadecenoyl)-glycine + CoA + H(+)

  Catalysed by HOGA1, AGXT, AGXT2, GLYATL2mitochondrial matrix

• all-trans-4-hydroxyretinoateReach from here →
  1. 4-hydroxy-2-oxoglutarate glyoxylate-lyase (pyruvate-forming)
  2. transport of pyruvate and retinoate (cytosol to extracellular)
  3. cytochrome P450 (retinoate)

  Catalysed by HOGA1, SLC16A4, SLC16A5, SLC16A3 and 4 moremitochondrial matrix, extracellular space, cytosolOne of 3 routes

• N-acetylcadaverineReach from here →
  1. 4-hydroxy-2-oxoglutarate glyoxylate-lyase (pyruvate-forming)
  2. transport of acetate and pyruvate (cytosol to extracellular)
  3. N-acetylcadaverine + H2O = cadaverine + acetate

  Catalysed by HOGA1, SLC16A4, SLC16A5, SLC16A3 and 3 moremitochondrial matrix, extracellular space, cytosol

• all-trans-retinoyl-1-O-(beta-D-glucuronate)Reach from here →
  1. 4-hydroxy-2-oxoglutarate glyoxylate-lyase (pyruvate-forming)
  2. transport of pyruvate and retinoate (cytosol to extracellular)
  3. glucuronosyltransferase (retinoate)

  Catalysed by HOGA1, SLC16A4, SLC16A5, SLC16A3 and 6 moremitochondrial matrix, extracellular space, cytosol, endoplasmic reticulum

• all-trans-18-hydroxyretinoateReach from here →
  1. 4-hydroxy-2-oxoglutarate glyoxylate-lyase (pyruvate-forming)
  2. transport of pyruvate and retinoate (cytosol to extracellular)
  3. unspecific monooxygenase (retinoate)

  Catalysed by HOGA1, SLC16A4, SLC16A5, SLC16A3 and 28 moremitochondrial matrix, extracellular space, cytosolOne of 2 routes

• a phenyl acetateReach from here →
  1. 4-hydroxy-2-oxoglutarate glyoxylate-lyase (pyruvate-forming)
  2. transport of acetate and pyruvate (cytosol to extracellular)
  3. a phenyl acetate + H2O = a phenol + acetate + H(+)

  Catalysed by HOGA1, SLC16A4, SLC16A5, SLC16A3 and 5 moremitochondrial matrix, extracellular space, cytosol

• N(2)-(1-hydroxy-2-oxoethyl)-dGTPReach from here →
  1. 4-hydroxy-2-oxoglutarate glyoxylate-lyase (pyruvate-forming)
  2. glycolate:NADP+ oxidoreductase
  3. N(2)-(1-hydroxy-2-oxoethyl)-dGTP + H2O = dGTP + glycolate + H(+)

  Catalysed by HOGA1, GRHPR, PARK7mitochondrial matrix, cytosol

• N(2)-(1-hydroxy-2-oxoethyl)-GMPReach from here →
  1. 4-hydroxy-2-oxoglutarate glyoxylate-lyase (pyruvate-forming)
  2. glycolate:NADP+ oxidoreductase
  3. N(2)-(1-hydroxy-2-oxoethyl)-GMP + H2O = glycolate + GMP + H(+)

  Catalysed by HOGA1, GRHPR, PARK7mitochondrial matrix, cytosol

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

  Catalysed by HOGA1, AGXT, AGXT2, GLYATL3mitochondrial matrix

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

  Catalysed by HOGA1, AGXT, AGXT2, GLYATL2mitochondrial matrix

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

  Catalysed by HOGA1, AGXT, AGXT2, GLYATL2mitochondrial matrix

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

  Catalysed by HOGA1, AGXT, AGXT2, GLYATL2mitochondrial matrix

• N(1)-(5-phospho-beta-D-ribosyl)glycinamideReach from here →
  1. 4-hydroxy-2-oxoglutarate glyoxylate-lyase (pyruvate-forming)
  2. L-Alanine:glyoxylate aminotransferase
  3. 5-phospho-beta-D-ribosylamine + glycine + ATP = N(1)-(5-phospho-beta-D-ribosyl)glycinamide + ADP + phosphate + H(+)

  Catalysed by HOGA1, AGXT, AGXT2, GARTmitochondrial matrix

• L-cystine-bis-glycineReach from here →
  1. 4-hydroxy-2-oxoglutarate glyoxylate-lyase (pyruvate-forming)
  2. L-Alanine:glyoxylate aminotransferase
  3. L-cystine-bis-glycine + 2 H2O = L-cystine + 2 glycine

  Catalysed by HOGA1, AGXT, AGXT2, DPEP1 and 1 moremitochondrial matrix

• glycyldehydrophenylalanineReach from here →
  1. 4-hydroxy-2-oxoglutarate glyoxylate-lyase (pyruvate-forming)
  2. L-Alanine:glyoxylate aminotransferase
  3. glycyldehydrophenylalanine + H2O = 2,3-didehydrophenylalanine + glycine

  Catalysed by HOGA1, AGXT, AGXT2, DPEP1mitochondrial matrix

• an N-(fatty acyl)-glycineReach from here →
  1. 4-hydroxy-2-oxoglutarate glyoxylate-lyase (pyruvate-forming)
  2. L-Alanine:glyoxylate aminotransferase
  3. a fatty acyl-CoA + glycine = an N-(fatty acyl)-glycine + CoA + H(+)

  Catalysed by HOGA1, AGXT, AGXT2, GLYATL2mitochondrial matrix

• N-(9Z,12Z-octadecadienoyl)-glycineReach from here →
  1. 4-hydroxy-2-oxoglutarate glyoxylate-lyase (pyruvate-forming)
  2. L-Alanine:glyoxylate aminotransferase
  3. (9Z,12Z)-octadecadienoyl-CoA + glycine = N-(9Z,12Z-octadecadienoyl)-glycine + CoA + H(+)

  Catalysed by HOGA1, AGXT, AGXT2, GLYATL2mitochondrial matrix

• (7Z)-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. carnitine O-palmitoyltransferase ((7Z)-octadecenoyl-CoA)

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

• CHEBI:15345Reach 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. Acetoacetate:CoA ligase (AMP-forming)

  Catalysed by HOGA1, CLYBL, AACSmitochondrial matrix, cytosolOne of 5 routes

• acetoneReach from here →
  1. 4-hydroxy-2-oxoglutarate glyoxylate-lyase (pyruvate-forming)
  2. transport of acetoacetate and pyruvate (cytosol to mitochondria)
  3. Acetoacetate carboxy-lyase (acetone-forming)

  Catalysed by HOGA1mitochondrial matrix, cytosol

• cholineReach 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:choline O-acetyltransferase

  Catalysed by HOGA1, CLYBL, CHATmitochondrial matrix, nucleus

• CHEBI:15380Reach 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. 5-aminolevulinate synthase (2-amino-3-oxoadipate)

  Catalysed by HOGA1, CLYBL, ALAS1, CPNE7mitochondrial matrixOne of 8 routes

• CHEBI:15477Reach 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. glycine N-acyltransferase (2-methylbutyryl-CoA)

  Catalysed by HOGA1, CLYBL, GLYAT, GLYATL2 and 1 moremitochondrial matrixOne of 2 routes

• 2-methylcrotonoyl-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 Tiglylcarnitine

  Catalysed by HOGA1, CLYBL, CPT1A, CPT1Bmitochondrial matrix, cytosolOne of 2 routes

• isobutyryl-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-methyl-pentanoyl-CoA)

  Catalysed by HOGA1, CLYBL, HADHA, ACAA2 and 1 moremitochondrial matrixOne of 3 routes

• 3-methylbut-2-enoyl-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. glycine-N-acyltransferase (3-methylcrotonoylglycine)

  Catalysed by HOGA1, CLYBL, GLYATmitochondrial matrix

• isovaleryl-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. glycine N-acyltransferase (isovalerylglycine)

  Catalysed by HOGA1, CLYBL, GLYAT, GLYATL2 and 1 moremitochondrial matrixOne of 3 routes

• arachidonoyl-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. Arachidonate:CoA ligase (AMP-forming)

  Catalysed by HOGA1, CLYBL, SLC27A2, ACSL4 and 6 moremitochondrial matrix, cytosolOne of 11 routes

• CHEBI:15519Reach 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. propanoyl-CoA C-acyltransferase

  Catalysed by HOGA1, CLYBL, ACAA1, SCP2 and 1 moremitochondrial matrix, peroxisomeOne of 2 routes

• palmitoyl-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. Palmitate:CoA ligase (AMP-forming)

  Catalysed by HOGA1, CLYBL, SLC27A2, ACSL4 and 6 moremitochondrial matrix, cytosolOne of 11 routes

• oleoyl-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 octadecenoyl-CoA into Mitochondrial Matrix

  Catalysed by HOGA1, CLYBL, CPT1A, CPT1C and 1 moremitochondrial matrix, cytosolOne of 11 routes

• phenylacetyl-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. Phenylacetate:CoA ligase

  Catalysed by HOGA1, CLYBLmitochondrial matrix, cytosolOne of 3 routes

• propionyl-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. Propanoate:CoA ligase (AMP-forming)

  Catalysed by HOGA1, CLYBL, ACSS3, ACSS2 and 1 moremitochondrial matrix, cytosolOne of 48 routes

• CHEBI:15570Reach from here →
  1. 4-hydroxy-2-oxoglutarate glyoxylate-lyase (pyruvate-forming)
  2. L-Alanine:glyoxylate aminotransferase
  3. transport of D-alanine and glycine (cytosol to extracellular)

  Catalysed by HOGA1, AGXT, AGXT2, SLC3A2 and 1 moremitochondrial matrix, extracellular space, cytosolOne of 3 routes

• L-leucineReach from here →
  1. 4-hydroxy-2-oxoglutarate glyoxylate-lyase (pyruvate-forming)
  2. L-Alanine:glyoxylate aminotransferase
  3. transport of glycine and leucine (cytosol to extracellular)

  Catalysed by HOGA1, AGXT, AGXT2, SLC7A5mitochondrial matrix, extracellular space, cytosolOne of 15 routes

• CHEBI:15611Reach from here →
  1. 4-hydroxy-2-oxoglutarate glyoxylate-lyase (pyruvate-forming)
  2. L-Alanine:glyoxylate aminotransferase
  3. S-Adenosyl-L-methionine:glycine N-methyltransferase

  Catalysed by HOGA1, AGXT, AGXT2, GNMTmitochondrial matrix, cytosol

• 10-formyltetrahydrofolic acidReach from here →
  1. 4-hydroxy-2-oxoglutarate glyoxylate-lyase (pyruvate-forming)
  2. transport of formate and pyruvate (cytosol to extracellular)
  3. Formate:tetrahydrofolate ligase (ADP-forming)

  Catalysed by HOGA1, SLC16A4, SLC16A5, SLC16A7 and 3 moremitochondrial matrix, extracellular space, cytosolOne of 2 routes

• CHEBI:15650Reach 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. N-acetyl-LTE4 to leukotriene E4 conversion

  Catalysed by HOGA1, CLYBL, ZDHHC11B, NAA10 and 30 moremitochondrial matrix, cytosolOne of 2 routes

• CHEBI:15699Reach from here →
  1. 4-hydroxy-2-oxoglutarate glyoxylate-lyase (pyruvate-forming)
  2. L-Alanine:glyoxylate aminotransferase
  3. transport of glycine and homoserine (cytosol to extracellular)

  Catalysed by HOGA1, AGXT, AGXT2, SLC7A5mitochondrial matrix, extracellular space, cytosolOne of 10 routes

• CHEBI:15727Reach 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. N-alpha-acetyltransferase 10, NatA catalytic subunit (acetylcarnosine)

  Catalysed by HOGA1, CLYBL, NAA10mitochondrial matrix, cytosol

• CHEBI:15746Reach 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. diamine N-acetyltransferase (N1-acetylspermine)

  Catalysed by HOGA1, CLYBL, SAT1, SAT2mitochondrial matrix, cytosolOne of 2 routes

• malonateReach 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. malonate(out) + (S)-malate(in) = malonate(in) + (S)-malate(out)

  Catalysed by HOGA1, CLYBL, SLC25A10mitochondrial matrix, inner side of the membrane, outer side of the membraneOne of 2 routes