4-hydroxy-2-oxoglutaric acid

Three steps

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

  Catalysed by HOGA1, AGXT, AGXT2, GSSmitochondrial matrix

• S-methylmercury-L-cysteineReach from here →
  1. 4-hydroxy-2-oxoglutarate glyoxylate-lyase (pyruvate-forming)
  2. N(1)-methylguanosine(37) in tRNA(Phe) + pyruvate + S-adenosyl-L-methionine = 4-demethylwyosine(37) in tRNA(Phe) + 5'-deoxyadenosine + L-methionine + CO2 + H2O
  3. S-methylmercury-L-cysteine(out) + L-methionine(in) = S-methylmercury-L-cysteine(in) + L-methionine(out)

  Catalysed by HOGA1, TYW1B, TYW1, SLC7A8mitochondrial matrix, inner side of the membrane, outer side of the membraneOne of 2 routes

• 2-(alpha-Hydroxypropyl)thiamine diphosphateReach from here →
  1. 4-hydroxy-2-oxoglutarate glyoxylate-lyase (pyruvate-forming)
  2. 2-oxobutanoate + L-alanine = (2S)-2-aminobutanoate + pyruvate
  3. branched chain keto acid dehydrogenase E1 (2-oxobutyrate)

  Catalysed by HOGA1, AGXT2, BCKDHA, BCKDHBmitochondrial matrix

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

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

• D-isoleucineReach from here →
  1. 4-hydroxy-2-oxoglutarate glyoxylate-lyase (pyruvate-forming)
  2. D-serine = pyruvate + NH4(+)
  3. D-isoleucine(out) + D-serine(in) = D-isoleucine(in) + D-serine(out)

  Catalysed by HOGA1, SRR, SLC7A10mitochondrial matrix, inner side of the membrane, outer side of the membrane

• N(omega),N('omega)-dimethyl-L-arginineReach from here →
  1. 4-hydroxy-2-oxoglutarate glyoxylate-lyase (pyruvate-forming)
  2. L-Alanine:glyoxylate aminotransferase
  3. N(omega),N('omega)-dimethyl-L-arginine + glyoxylate = 5-(3,3'-dimethylguanidino)-2-oxopentanoate + glycine

  Catalysed by HOGA1, AGXT, AGXT2mitochondrial matrixOne of 4 routes

• 5,6,7,8-tetrahydrofolic acidReach from here →
  1. 4-hydroxy-2-oxoglutarate glyoxylate-lyase (pyruvate-forming)
  2. L-Alanine:glyoxylate aminotransferase
  3. 5,10-Methylenetetrahydrofolate:glycine hydroxymethyltransferase

  Catalysed by HOGA1, AGXT, AGXT2, SHMT1mitochondrial matrix, cytosolOne of 2 routes

• (5Z)-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. acetyl-CoA C-acyltransferase (3-oxomyrist-7-enoyl-CoA)

  Catalysed by HOGA1, CLYBL, HADHA, ACAA2 and 1 moremitochondrial matrixOne of 2 routes

• (S)-lactoyl-AMPReach from here →
  1. 4-hydroxy-2-oxoglutarate glyoxylate-lyase (pyruvate-forming)
  2. (S)-lactate + NAD(+) = pyruvate + NADH + H(+)
  3. (S)-lactate + ATP + H(+) = (S)-lactoyl-AMP + diphosphate

  Catalysed by HOGA1, LDHA, LDHB, LDHC and 4 moremitochondrial matrix

• (S)-lactoyl-CoAReach from here →
  1. 4-hydroxy-2-oxoglutarate glyoxylate-lyase (pyruvate-forming)
  2. (S)-lactate + NAD(+) = pyruvate + NADH + H(+)
  3. (S)-lactate + ATP + CoA = (S)-lactoyl-CoA + AMP + diphosphate

  Catalysed by HOGA1, LDHA, LDHB, LDHC and 3 moremitochondrial matrix

• N-acetylneuraminate 9-phosphateReach from here →
  1. 4-hydroxy-2-oxoglutarate glyoxylate-lyase (pyruvate-forming)
  2. pyruvate kinase (dTDP)
  3. N-acetylneuraminate synthase

  Catalysed by HOGA1, PKM, PKLR, NANSmitochondrial matrix, cytosol

• (11Z)-docosenoyl-CoA(4-)Reach 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 (cis-cetoleic acid)

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

• (7Z)-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 ((7Z)-tetradecenoic acid)

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

• (4Z,7Z,10Z,13Z)-hexadecatetraenoyl-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 (C18:4->C16:4), Mitochondrial

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

• (7Z,10Z)-hexadecadienoyl-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 Trans (C18:2->C16:2), Mitochondrial

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

• (4Z,7Z)-decadienoyl-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 (C12:3->C10:2), Mitochondrial

  Catalysed by HOGA1, CLYBL, ECHS1, ACAA2 and 1 moremitochondrial matrixOne of 2 routes

• (5Z)-octenoyl-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 (C10:2->C8:1), Mitochondrial

  Catalysed by HOGA1, CLYBL, ECHS1, ACAA2 and 1 moremitochondrial matrixOne of 2 routes

• CHEBI:240943Reach from here →
  1. 4-hydroxy-2-oxoglutarate glyoxylate-lyase (pyruvate-forming)
  2. L-Alanine:glyoxylate aminotransferase
  3. glycine N-acyltransferase (2-methylbutyryl-CoA)

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

• a quinolReach 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 + a quinone = enol-oxaloacetate + a quinol

  Catalysed by HOGA1, CLYBL, SDHB, SDHAmitochondrial matrix

• phenylacetylglycineReach from here →
  1. 4-hydroxy-2-oxoglutarate glyoxylate-lyase (pyruvate-forming)
  2. L-Alanine:glyoxylate aminotransferase
  3. phenylacetyl-CoA:glycine N-phenylacetyltransferase

  Catalysed by HOGA1, AGXT, AGXT2, GLYATmitochondrial matrix, cytosolOne of 3 routes

• CHEBI:27540Reach 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 (hexanoic acid)

  Catalysed by HOGA1, CLYBL, ACSM4, ACSM1 and 4 moremitochondrial matrix, cytosolOne of 6 routes

• selenomethionineReach from here →
  1. 4-hydroxy-2-oxoglutarate glyoxylate-lyase (pyruvate-forming)
  2. 2-oxobutanoate + L-alanine = (2S)-2-aminobutanoate + pyruvate
  3. Selenomethionine methanethiol-lyase (deaminating)

  Catalysed by HOGA1, AGXT2, CTHmitochondrial matrix, cytosol

• CHEBI:27760Reach from here →
  1. 4-hydroxy-2-oxoglutarate glyoxylate-lyase (pyruvate-forming)
  2. 2-oxobutanoate + L-alanine = (2S)-2-aminobutanoate + pyruvate
  3. Selenocystathionine Lysteine-lyase (deaminating)

  Catalysed by HOGA1, AGXT2, CTHmitochondrial matrix, cytosol

• elaidic acidReach 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 (elaidate)

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

• 3-mercaptolactic acidReach from here →
  1. 4-hydroxy-2-oxoglutarate glyoxylate-lyase (pyruvate-forming)
  2. 2-oxo-3-sulfanylpropanoate + [thioredoxin]-dithiol = [thioredoxin]-disulfide + hydrogen sulfide + pyruvate + H(+)
  3. 3-Mercaptolactate:NAD+ oxidoreductase

  Catalysed by HOGA1, MPST, LDHB, LDHC and 2 moremitochondrial matrix, cytosol

• 2-oxopentanoateReach from here →
  1. 4-hydroxy-2-oxoglutarate glyoxylate-lyase (pyruvate-forming)
  2. N(omega),N(omega)-dimethyl-L-arginine + pyruvate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + L-alanine
  3. 2-oxopentanoate + N(omega),N(omega)-dimethyl-L-arginine = 5-(3,3-dimethylguanidino)-2-oxopentanoate + L-2-aminopentanoate

  Catalysed by HOGA1, AGXT2mitochondrial matrix

• CHEBI:28666Reach from here →
  1. 4-hydroxy-2-oxoglutarate glyoxylate-lyase (pyruvate-forming)
  2. L-Alanine:glyoxylate aminotransferase
  3. LTD4 hydrolysis

  Catalysed by HOGA1, AGXT, AGXT2mitochondrial matrix, cytosol

• CHEBI:28701Reach 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:acetyl-CoA C-acyltransferase

  Catalysed by HOGA1, CLYBL, ACAA1, SCP2 and 1 moremitochondrial matrix, peroxisomeOne of 7 routes

• 17alpha-hydroxypregnenoloneReach from here →
  1. 4-hydroxy-2-oxoglutarate glyoxylate-lyase (pyruvate-forming)
  2. transport of acetate and pyruvate (cytosol to extracellular)
  3. cytochrome P450 (17alpha-hydroxypregnenolone)

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

• pyridoxine 5'-phosphateReach from here →
  1. 4-hydroxy-2-oxoglutarate glyoxylate-lyase (pyruvate-forming)
  2. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  3. L-selenocysteine selenide-lyase (L-alanine-forming)

  Catalysed by HOGA1, AGXT2, SCLYmitochondrial matrix, cytosol

• N-glycoloylneuraminateReach from here →
  1. 4-hydroxy-2-oxoglutarate glyoxylate-lyase (pyruvate-forming)
  2. N-Acetylneuraminate pyruvate-lyase (N-acetyl-D-mannosamine-forming)
  3. CMP-N-acetylneuraminate monooxygenase (N-acetylneuraminate)

  Catalysed by HOGA1, NPLmitochondrial matrix, cytosol

• a carboxylateReach from here →
  1. 4-hydroxy-2-oxoglutarate glyoxylate-lyase (pyruvate-forming)
  2. a 3-acylpyruvate + H2O = a carboxylate + pyruvate + H(+)
  3. a 3-acylpyruvate + H2O = a carboxylate + pyruvate + H(+)

  Catalysed by HOGA1, FAHD1mitochondrial matrix

• Gly-tRNA(Gly)Reach from here →
  1. 4-hydroxy-2-oxoglutarate glyoxylate-lyase (pyruvate-forming)
  2. L-Alanine:glyoxylate aminotransferase
  3. glycine:tRNA(Gly) ligase (AMP-forming)

  Catalysed by HOGA1, AGXT, AGXT2, GARS1mitochondrial matrix, cytosol

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

  Catalysed by HOGA1, AGXT, AGXT2, SLC6A9 and 1 moremitochondrial matrix, extracellular space, cytosolOne of 10 routes

• glycocholateReach from here →
  1. 4-hydroxy-2-oxoglutarate glyoxylate-lyase (pyruvate-forming)
  2. L-Alanine:glyoxylate aminotransferase
  3. bile acid-CoA: amino acid N-acyltransferase

  Catalysed by HOGA1, AGXT, AGXT2, BAATmitochondrial matrix, peroxisomeOne of 3 routes

• hydrogen sulfideReach from here →
  1. 4-hydroxy-2-oxoglutarate glyoxylate-lyase (pyruvate-forming)
  2. 2-oxo-3-sulfanylpropanoate + [thioredoxin]-dithiol = [thioredoxin]-disulfide + hydrogen sulfide + pyruvate + H(+)
  3. 2-oxo-3-sulfanylpropanoate + [thioredoxin]-dithiol = [thioredoxin]-disulfide + hydrogen sulfide + pyruvate + H(+)

  Catalysed by HOGA1, MPSTmitochondrial matrix

• L-aspartateReach from here →
  1. 4-hydroxy-2-oxoglutarate glyoxylate-lyase (pyruvate-forming)
  2. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  3. oxaloacetate + L-alanine = L-aspartate + pyruvate

  Catalysed by HOGA1, AGXT2mitochondrial matrixOne of 8 routes

• citric acidReach from here →
  1. 4-hydroxy-2-oxoglutarate glyoxylate-lyase (pyruvate-forming)
  2. Glyoxylate:oxygen oxidoreductase
  3. Mitochondrial Carrier (Mc) Tcdb:2.A.29.7.2

  Catalysed by HOGA1, HAO1, SLC25A1mitochondrial matrix, peroxisome, cytosolOne of 12 routes

• isocitric acidReach from here →
  1. 4-hydroxy-2-oxoglutarate glyoxylate-lyase (pyruvate-forming)
  2. Glyoxylate:oxygen oxidoreductase
  3. Mitochondrial Carrier (Mc) Tcdb:2.A.29.7.2

  Catalysed by HOGA1, HAO1, SLC25A1mitochondrial matrix, peroxisome, cytosolOne of 8 routes

• cis-aconitic acidReach from here →
  1. 4-hydroxy-2-oxoglutarate glyoxylate-lyase (pyruvate-forming)
  2. Glyoxylate:oxygen oxidoreductase
  3. Mitochondrial Carrier (Mc) Tcdb:2.A.29.7.2

  Catalysed by HOGA1, HAO1, SLC25A1mitochondrial matrix, peroxisome, cytosolOne of 8 routes

• thiosulfateReach 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 malate and thiosulfate (cytosol to mitochondria)

  Catalysed by HOGA1, CLYBL, SLC25A10mitochondrial matrix, cytosolOne of 4 routes

• 4-methylthio-2-oxobutanoic acidReach from here →
  1. 4-hydroxy-2-oxoglutarate glyoxylate-lyase (pyruvate-forming)
  2. N(1)-methylguanosine(37) in tRNA(Phe) + pyruvate + S-adenosyl-L-methionine = 4-demethylwyosine(37) in tRNA(Phe) + 5'-deoxyadenosine + L-methionine + CO2 + H2O
  3. L-Methionine:oxygen oxidoreductase (deaminating)

  Catalysed by HOGA1, TYW1B, TYW1, IL4I1mitochondrial matrix, cytosol

• 2-oxohexanoateReach from here →
  1. 4-hydroxy-2-oxoglutarate glyoxylate-lyase (pyruvate-forming)
  2. N(omega),N(omega)-dimethyl-L-arginine + pyruvate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + L-alanine
  3. 2-oxohexanoate + N(omega),N(omega)-dimethyl-L-arginine = L-2-aminohexanoate + 5-(3,3-dimethylguanidino)-2-oxopentanoate

  Catalysed by HOGA1, AGXT2mitochondrial matrix

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

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

• L-alpha-aminobutyric acidReach from here →
  1. 4-hydroxy-2-oxoglutarate glyoxylate-lyase (pyruvate-forming)
  2. 2-oxobutanoate + L-alanine = (2S)-2-aminobutanoate + pyruvate
  3. glutamic-pyruvic transaminase ((S)-2-aminobutanoate)

  Catalysed by HOGA1, AGXT2, GPTmitochondrial matrix, cytosol

• 5-aminolevulinateReach from here →
  1. 4-hydroxy-2-oxoglutarate glyoxylate-lyase (pyruvate-forming)
  2. L-Alanine:glyoxylate aminotransferase
  3. succinyl-CoA + glycine + H(+) = 5-aminolevulinate + CO2 + CoA

  Catalysed by HOGA1, AGXT, AGXT2, ALAS1 and 1 moremitochondrial matrix

• glycochenodeoxycholateReach from here →
  1. 4-hydroxy-2-oxoglutarate glyoxylate-lyase (pyruvate-forming)
  2. L-Alanine:glyoxylate aminotransferase
  3. chenodeoxycholoyl-CoA + glycine = glycochenodeoxycholate + CoA + H(+)

  Catalysed by HOGA1, AGXT, AGXT2, BAATmitochondrial matrix

• glycochenodeoxycholic acidReach from here →
  1. 4-hydroxy-2-oxoglutarate glyoxylate-lyase (pyruvate-forming)
  2. L-Alanine:glyoxylate aminotransferase
  3. bile acid-CoA: amino acid N-acyltransferase

  Catalysed by HOGA1, AGXT, AGXT2, BAATmitochondrial matrix, peroxisomeOne of 3 routes

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

  Catalysed by HOGA1, AGXT, AGXT2, AADATmitochondrial matrixOne of 21 routes

• glycolithocholic acidReach from here →
  1. 4-hydroxy-2-oxoglutarate glyoxylate-lyase (pyruvate-forming)
  2. L-Alanine:glyoxylate aminotransferase
  3. bile acid-CoA:amino acid N-acyltransferase (glycine)

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