4-hydroxy-2-oxoglutaric acid

Three steps

• L-2-amino-3-oxobutanoic acidReach from here →
  1. 4-hydroxy-2-oxoglutarate glyoxylate-lyase (pyruvate-forming)
  2. L-Alanine:glyoxylate aminotransferase
  3. Acetyl-CoA:glycine C-acetyltransferase

  Catalysed by HOGA1, AGXT, AGXT2, GCATmitochondrial matrix

• (3S)-3-hydroxy-3-methylglutaryl-CoAReach from here →
  1. 4-hydroxy-2-oxoglutarate glyoxylate-lyase (pyruvate-forming)
  2. transport of acetoacetate and pyruvate (cytosol to mitochondria)
  3. (3S)-3-hydroxy-3-methylglutaryl-CoA = acetoacetate + acetyl-CoA

  Catalysed by HOGA1, HMGCL, HMGCLL1mitochondrial matrix, cytosol

• 13-cis-retinalReach from here →
  1. 4-hydroxy-2-oxoglutarate glyoxylate-lyase (pyruvate-forming)
  2. transport of pyruvate and retinoate (cytosol to extracellular)
  3. retinal dehydrogenase (13-cis-retinal)

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

• L-ornithineReach from here →
  1. 4-hydroxy-2-oxoglutarate glyoxylate-lyase (pyruvate-forming)
  2. L-Alanine:glyoxylate aminotransferase
  3. L-Arginine:glycine amidinotransferase

  Catalysed by HOGA1, AGXT, AGXT2, GATMmitochondrial matrix, cytosolOne of 12 routes

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

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

• gamma-Glu-AlaReach from here →
  1. 4-hydroxy-2-oxoglutarate glyoxylate-lyase (pyruvate-forming)
  2. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  3. gamma-glutamyltransferase (alanine)

  Catalysed by HOGA1, AGXT2, GGT1, GGT5 and 4 moremitochondrial matrix, extracellular space, cytosol

• S(8)-aminomethyldihydrolipoamideReach from here →
  1. 4-hydroxy-2-oxoglutarate glyoxylate-lyase (pyruvate-forming)
  2. L-Alanine:glyoxylate aminotransferase
  3. dihydrolipoyl dehydrogenase ([protein]-N6-(lipoyl)lysine)

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

• taurineReach 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. bile acid-CoA:amino acid N-acyltransferase (taurodeoxycholate)

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

• acetoacetyl-CoAReach from here →
  1. 4-hydroxy-2-oxoglutarate glyoxylate-lyase (pyruvate-forming)
  2. transport of acetoacetate and pyruvate (cytosol to mitochondria)
  3. acetoacetate + ATP + CoA = acetoacetyl-CoA + AMP + diphosphate

  Catalysed by HOGA1, AACSmitochondrial matrix, cytosolOne of 2 routes

• (S)-malyl-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. (S)-malate + ATP + CoA = (S)-malyl-CoA + ADP + phosphate

  Catalysed by HOGA1, CLYBL, SUCLG1, SUCLA2mitochondrial matrix

• (R)-methylmalonyl-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. aldehyde dehydrogenase (2-methyl-3-oxopropanoate)

  Catalysed by HOGA1, CLYBL, ALDH6A1mitochondrial matrixOne of 3 routes

• (S)-methylmalonyl-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. (S)-methylmalonyl-CoA hydrolase

  Catalysed by HOGA1, CLYBLmitochondrial matrix

• 3'-dephospho-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. acid phosphatase 2, lysosomal (CoA)

  Catalysed by HOGA1, CLYBL, ACP2mitochondrial matrix, lysosomeOne of 2 routes

• (2E)-dodecenoyl-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. (2E)-dodecenoyl-CoA formation

  Catalysed by HOGA1, CLYBLmitochondrial matrixOne of 3 routes

• 4,8,12-trimethyltridecanoyl-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. propanoyl-CoA C-acyltransferase (3-ketopristanoyl-CoA)

  Catalysed by HOGA1, CLYBL, ACAA1, MYO5Bmitochondrial matrix, peroxisomeOne of 2 routes

• (6Z,9Z,12Z)-octadecatrienoyl-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 (gamma-linolenate)

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

• butanoyl-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. Butanoate:CoA ligase (AMP-forming)

  Catalysed by HOGA1, CLYBL, ACSM4, ACSM1 and 4 moremitochondrial matrix, cytosolOne of 11 routes

• dodecanoyl-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 (lauric acid)

  Catalysed by HOGA1, CLYBL, SLC27A2, ACSL4 and 6 moremitochondrial matrix, cytosolOne of 9 routes

• glutaryl-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 Glutaryl Carnitine

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

• eicosanoyl-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 (eicosanoate)

  Catalysed by HOGA1, CLYBL, SLC27A2, ACSL4 and 6 moremitochondrial matrix, cytosolOne of 12 routes

• itaconyl-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. Itaconate:CoA ligase (GDP-forming)

  Catalysed by HOGA1, CLYBL, SUCLG1, SUCLG2 and 1 moremitochondrial matrixOne of 2 routes

• (9Z,12Z)-octadecadienoyl-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 ((6Z,9Z)-octadecadienoic acid)

  Catalysed by HOGA1, CLYBL, SLC27A2, ACSL4 and 6 moremitochondrial matrix, cytosolOne of 13 routes

• tetradecanoyl-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 (myristic acid)

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

• octanoyl-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. Octanoyl-CoA:L-carnitine O-octanoyltransferase

  Catalysed by HOGA1, CLYBL, CROTmitochondrial matrixOne of 20 routes

• phytanoyl-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. phytanate-CoA ligase

  Catalysed by HOGA1, CLYBLmitochondrial matrix, cytosolOne of 5 routes

• propanoyl-CoAReach 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. 2-methyl-3-oxopropanoate + NAD(+) + CoA + H2O = propanoyl-CoA + hydrogencarbonate + NADH + H(+)

  Catalysed by HOGA1, AGXT2, ALDH6A1mitochondrial matrix

• octadecanoyl-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 (stearate)

  Catalysed by HOGA1, CLYBL, SLC27A2, ACSL4 and 5 moremitochondrial matrix, cytosolOne of 15 routes

• prostaglandin E1Reach from here →
  1. 4-hydroxy-2-oxoglutarate glyoxylate-lyase (pyruvate-forming)
  2. (S)-lactate + NAD(+) = pyruvate + NADH + H(+)
  3. prostaglandin E1(out) + 2 (S)-lactate(in) = prostaglandin E1(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

• prostaglandin F2alphaReach from here →
  1. 4-hydroxy-2-oxoglutarate glyoxylate-lyase (pyruvate-forming)
  2. (S)-lactate + NAD(+) = pyruvate + NADH + H(+)
  3. prostaglandin F2alpha(out) + 2 (S)-lactate(in) = prostaglandin F2alpha(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

• prostaglandin H2Reach from here →
  1. 4-hydroxy-2-oxoglutarate glyoxylate-lyase (pyruvate-forming)
  2. (S)-lactate + NAD(+) = pyruvate + NADH + H(+)
  3. prostaglandin H2(out) + 2 (S)-lactate(in) = prostaglandin H2(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

• prostaglandin D2Reach from here →
  1. 4-hydroxy-2-oxoglutarate glyoxylate-lyase (pyruvate-forming)
  2. (S)-lactate + NAD(+) = pyruvate + NADH + H(+)
  3. prostaglandin D2(out) + 2 (S)-lactate(in) = prostaglandin D2(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

• D-alanineReach from here →
  1. 4-hydroxy-2-oxoglutarate glyoxylate-lyase (pyruvate-forming)
  2. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  3. D-alanine(out) + L-alanine(in) = D-alanine(in) + L-alanine(out)

  Catalysed by HOGA1, AGXT2, SLC7A10mitochondrial matrix, inner side of the membrane, outer side of the membraneOne of 2 routes

• sarcosineReach from here →
  1. 4-hydroxy-2-oxoglutarate glyoxylate-lyase (pyruvate-forming)
  2. L-Alanine:glyoxylate aminotransferase
  3. sarcosine + O2 + H2O = formaldehyde + glycine + H2O2

  Catalysed by HOGA1, AGXT, AGXT2, PIPOXmitochondrial matrixOne of 2 routes

• (6S)-5,6,7,8-tetrahydrofolateReach from here →
  1. 4-hydroxy-2-oxoglutarate glyoxylate-lyase (pyruvate-forming)
  2. L-Alanine:glyoxylate aminotransferase
  3. (6R)-5,10-methylene-5,6,7,8-tetrahydrofolate + glycine + H2O = (6S)-5,6,7,8-tetrahydrofolate + L-serine

  Catalysed by HOGA1, AGXT, AGXT2, SHMT1 and 1 moremitochondrial matrix

• N-acetyl-D-glucosamine 6-phosphateReach from here →
  1. 4-hydroxy-2-oxoglutarate glyoxylate-lyase (pyruvate-forming)
  2. transport of acetate and pyruvate (cytosol to extracellular)
  3. N-acetyl-D-glucosamine 6-phosphate + H2O = D-glucosamine 6-phosphate + acetate

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

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

  Catalysed by HOGA1, AGXT, AGXT2, GLYATL3 and 2 moremitochondrial matrix

• guanidinoacetateReach from here →
  1. 4-hydroxy-2-oxoglutarate glyoxylate-lyase (pyruvate-forming)
  2. L-Alanine:glyoxylate aminotransferase
  3. L-Arginine:glycine amidinotransferase

  Catalysed by HOGA1, AGXT, AGXT2, GATMmitochondrial matrix, cytosolOne of 2 routes

• sphing-4-enineReach 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. acyl-CoA:sphingosine N-acyltransferase

  Catalysed by HOGA1, CLYBL, ASAH1, ACER2 and 2 moremitochondrial matrix, Golgi apparatusOne of 2 routes

• D-threonineReach from here →
  1. 4-hydroxy-2-oxoglutarate glyoxylate-lyase (pyruvate-forming)
  2. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  3. D-threonine(out) + L-alanine(in) = D-threonine(in) + L-alanine(out)

  Catalysed by HOGA1, AGXT2, SLC7A10mitochondrial matrix, inner side of the membrane, outer side of the membraneOne of 2 routes

• CMP-N-acetyl-beta-neuraminateReach from here →
  1. 4-hydroxy-2-oxoglutarate glyoxylate-lyase (pyruvate-forming)
  2. N-Acetylneuraminate pyruvate-lyase (N-acetyl-D-mannosamine-forming)
  3. CTP:N-acetylneuraminate cytidylyltransferase

  Catalysed by HOGA1, NPL, CMASmitochondrial matrix, cytosol, nucleusOne of 2 routes

• spermidineReach 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-acetylspermidine)

  Catalysed by HOGA1, CLYBL, SAT1, SAT2mitochondrial matrix, cytosolOne of 3 routes

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

  Catalysed by HOGA1, AGXT, AGXT2, SLC7A5mitochondrial matrix, extracellular space, cytosolOne of 13 routes

• glutathioneReach from here →
  1. 4-hydroxy-2-oxoglutarate glyoxylate-lyase (pyruvate-forming)
  2. L-Alanine:glyoxylate aminotransferase
  3. gamma-L-glutamyl-L-cysteine + glycine + ATP = glutathione + ADP + phosphate + H(+)

  Catalysed by HOGA1, AGXT, AGXT2, GSSmitochondrial matrix

• L-threonineReach from here →
  1. 4-hydroxy-2-oxoglutarate glyoxylate-lyase (pyruvate-forming)
  2. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  3. L-threonine(out) + L-alanine(in) = L-threonine(in) + L-alanine(out)

  Catalysed by HOGA1, AGXT2, SLC7A10mitochondrial matrix, inner side of the membrane, outer side of the membraneOne of 5 routes

• L-kynurenineReach from here →
  1. 4-hydroxy-2-oxoglutarate glyoxylate-lyase (pyruvate-forming)
  2. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  3. L-kynurenine + H2O = anthranilate + L-alanine + H(+)

  Catalysed by HOGA1, AGXT2, KYNUmitochondrial matrix

• beta-alanineReach from here →
  1. 4-hydroxy-2-oxoglutarate glyoxylate-lyase (pyruvate-forming)
  2. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  3. beta-alanine(out) + L-alanine(in) = beta-alanine(in) + L-alanine(out)

  Catalysed by HOGA1, AGXT2, SLC7A10mitochondrial matrix, inner side of the membrane, outer side of the membraneOne of 2 routes

• leukotriene C4Reach from here →
  1. 4-hydroxy-2-oxoglutarate glyoxylate-lyase (pyruvate-forming)
  2. L-Alanine:glyoxylate aminotransferase
  3. leukotriene C4 hydrolysis

  Catalysed by HOGA1, AGXT, AGXT2mitochondrial matrix, cytosol

• 2-hydroxy-3-oxopropanoateReach from here →
  1. 4-hydroxy-2-oxoglutarate glyoxylate-lyase (pyruvate-forming)
  2. L-Serine:pyruvate aminotransferase
  3. hydroxypyruvate aldose-ketose-isomerase

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

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

  Catalysed by HOGA1, AGXT, AGXT2mitochondrial matrix, extracellular space, cytosolOne of 14 routes

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

  Catalysed by HOGA1, AGXT, AGXT2, SLC7A5mitochondrial matrix, extracellular space, cytosolOne of 19 routes