glyoxylic acid

Three steps

• S-adenosylglutathioneReach from here →
  1. L-Alanine:glyoxylate aminotransferase
  2. gamma-L-glutamyl-L-cysteine + glycine + ATP = glutathione + ADP + phosphate + H(+)
  3. apo-[alkylcobalamin reductase] + adenosylcob(III)alamin + glutathione = S-adenosylglutathione + cob(I)alamin-[alkylcobalamin reductase] + H(+)

  Catalysed by AGXT, AGXT2, GSS, MMACHCmitochondrial matrix

• N(2)-formyl-N(1)-(5-phospho-beta-D-ribosyl)glycinamideReach from here →
  1. L-Alanine:glyoxylate aminotransferase
  2. 5-phospho-beta-D-ribosylamine + glycine + ATP = N(1)-(5-phospho-beta-D-ribosyl)glycinamide + ADP + phosphate + H(+)
  3. N(1)-(5-phospho-beta-D-ribosyl)glycinamide + (6R)-10-formyltetrahydrofolate = N(2)-formyl-N(1)-(5-phospho-beta-D-ribosyl)glycinamide + (6S)-5,6,7,8-tetrahydrofolate + H(+)

  Catalysed by AGXT, AGXT2, GARTmitochondrial matrix

• 2-formamido-N(1)-(5-O-phospho-beta-D-ribosyl)acetamidineReach from here →
  1. L-Alanine:glyoxylate aminotransferase
  2. transport of glutamine and glycine (cytosol to extracellular)
  3. N(2)-formyl-N(1)-(5-phospho-beta-D-ribosyl)glycinamide + L-glutamine + ATP + H2O = 2-formamido-N(1)-(5-O-phospho-beta-D-ribosyl)acetamidine + L-glutamate + ADP + phosphate + H(+)

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

• kinetensinReach from here →
  1. L-Alanine:glyoxylate aminotransferase
  2. transport of glycine and phenylalanine (cytosol to extracellular)
  3. Chymase

  Catalysed by AGXT, AGXT2, SLC7A5, CMA1mitochondrial matrix, extracellular space, cytosolOne of 3 routes

• 1-(10Z-heptadecenoyl)-2-(9Z,12Z-octadecadienoyl)-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 ((6Z,9Z)-octadecadienoic acid)
  3. 1-(10Z-heptadecenoyl)-sn-glycero-3-phosphoethanolamine + (9Z,12Z)-octadecadienoyl-CoA = 1-(10Z-heptadecenoyl)-2-(9Z,12Z-octadecadienoyl)-sn-glycero-3-phosphoethanolamine + CoA

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

• cholesteryl (7Z)-octadecenoateReach from here →
  1. acetyl-CoA[m] + glyoxalate[m] + H2O[m] = CoA[m] + H+[m] + malate[m]
  2. carnitine O-palmitoyltransferase ((7Z)-octadecenoyl-CoA)
  3. (7Z)-octadecenoyl-CoA + cholesterol = cholesteryl (7Z)-octadecenoate + CoA

  Catalysed by CLYBL, CPT1A, CPT1C, CPT1B and 1 moremitochondrial matrix, cytosol

• acetaldehydeReach from here →
  1. L-Alanine:glyoxylate aminotransferase
  2. transport of glycine and threonine (cytosol to extracellular)
  3. L-threonine acetaldehyde-lyase (glycine-forming)

  Catalysed by AGXT, AGXT2, SLC3A2, SLC7A5 and 1 moremitochondrial matrix, extracellular space, cytosol

• acetoneReach from here →
  1. L-Alanine:glyoxylate aminotransferase
  2. transport of acetoacetate and pyruvate (cytosol to mitochondria)
  3. Acetoacetate carboxy-lyase (acetone-forming)

  Catalysed by AGXT, AGXT2mitochondrial matrix, cytosol

• acetylcholineReach from here →
  1. L-Alanine:glyoxylate aminotransferase
  2. gamma-L-glutamyl-L-cysteine:glycine ligase (ADP-forming)
  3. transport of acetylcholine and GSH (cytosol to extracellular)

  Catalysed by AGXT, AGXT2, GSS, SLCO1A2 and 1 moremitochondrial matrix, cytosol, extracellular spaceOne of 5 routes

• 4a-hydroxytetrahydrobiopterinReach from here →
  1. L-Alanine:glyoxylate aminotransferase
  2. transport of glycine and tryptophan (cytosol to extracellular)
  3. L-tryptophan,tetrahydrobiopterin:oxygen oxidoreductase(5-hydroxylating)

  Catalysed by AGXT, AGXT2, SLC7A5, TPH1 and 1 moremitochondrial matrix, extracellular space, cytosolOne of 3 routes

• CHEBI:15467Reach from here →
  1. acetyl-CoA[m] + glyoxalate[m] + H2O[m] = CoA[m] + H+[m] + malate[m]
  2. Acetoacetate:CoA ligase (AMP-forming)
  3. 3-hydroxy-3-methylglutaryl-CoA synthase (acetoacetyl-CoA)

  Catalysed by CLYBL, AACS, HMGCS1mitochondrial matrix, cytosolOne of 3 routes

• D-threo-isocitrateReach from here →
  1. Glyoxylate:oxygen oxidoreductase
  2. Mitochondrial Carrier (Mc) Tcdb:2.A.29.7.2
  3. citrate = D-threo-isocitrate

  Catalysed by HAO1, SLC25A1, ACO1, ACO2peroxisome, mitochondrial matrix, cytosolOne of 3 routes

• (6R)-5,10-methylene-5,6,7,8-tetrahydrofolateReach 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. (6R)-5,10-methylene-5,6,7,8-tetrahydrofolate + glycine + H2O = (6S)-5,6,7,8-tetrahydrofolate + L-serine

  Catalysed by AGXT, AGXT2, SHMT1, SHMT2mitochondrial matrixOne of 2 routes

• 10-formyltetrahydrofolic acidReach from here →
  1. L-Alanine:glyoxylate aminotransferase
  2. 5,10-Methylenetetrahydrofolate:glycine hydroxymethyltransferase
  3. Formate:tetrahydrofolate ligase (ADP-forming)

  Catalysed by AGXT, AGXT2, SHMT1, MTHFD1mitochondrial matrix, cytosolOne of 5 routes

• 5-methyltetrahydrofolic acidReach from here →
  1. L-Alanine:glyoxylate aminotransferase
  2. 5,10-Methylenetetrahydrofolate:glycine hydroxymethyltransferase
  3. histamine N-methyltransferase (SAM)

  Catalysed by AGXT, AGXT2, SHMT1, HNMTmitochondrial matrix, cytosol

• (4aS,6R)-4a-hydroxy-L-erythro-5,6,7,8-tetrahydrobiopterinReach from here →
  1. L-Alanine:glyoxylate aminotransferase
  2. transport of glycine and tryptophan (cytosol to extracellular)
  3. (6R)-L-erythro-5,6,7,8-tetrahydrobiopterin + L-tryptophan + O2 = 5-hydroxy-L-tryptophan + (4aS,6R)-4a-hydroxy-L-erythro-5,6,7,8-tetrahydrobiopterin

  Catalysed by AGXT, AGXT2, SLC7A5, TPH1 and 1 moremitochondrial matrix, extracellular space, cytosolOne of 3 routes

• trans-aconitateReach from here →
  1. Glyoxylate:oxygen oxidoreductase
  2. Mitochondrial Carrier (Mc) Tcdb:2.A.29.7.2
  3. trans-aconitate(in) + citrate(out) = trans-aconitate(out) + citrate(in)

  Catalysed by HAO1, SLC25A1peroxisome, mitochondrial matrix, cytosol, inner side of the membrane, outer side of the membraneOne of 2 routes

• betaine aldehydeReach from here →
  1. acetyl-CoA[m] + glyoxalate[m] + H2O[m] = CoA[m] + H+[m] + malate[m]
  2. Acetyl-CoA:choline O-acetyltransferase
  3. choline dehydrogenase (choline)

  Catalysed by CLYBL, CHAT, CHDHmitochondrial matrix, nucleus, cytosolOne of 3 routes

• trimethylenediamineReach from here →
  1. L-Alanine:glyoxylate aminotransferase
  2. transport of glycine and lysine (cytosol to extracellular)
  3. deoxyhypusine synthase (1,3-diaminopropane)

  Catalysed by AGXT, AGXT2, SLC7A6, DHPSmitochondrial matrix, extracellular space, cytosolOne of 3 routes

• hexadecanoic acidReach from here →
  1. acetyl-CoA[m] + glyoxalate[m] + H2O[m] = CoA[m] + H+[m] + malate[m]
  2. Carnitine O-Palmitoyltransferase
  3. Utilized Transport

  Catalysed by CLYBL, CPT1A, CPT1C, CPT1Bmitochondrial matrix, cytosol, endoplasmic reticulumOne of 4 routes

• CHEBI:15765Reach from here →
  1. L-Alanine:glyoxylate aminotransferase
  2. transport of glycine and tyrosine (cytosol to extracellular)
  3. L-Tyrosine,tetrahydrobiopterin:oxygen oxidoreductase (3-hydroxylating)

  Catalysed by AGXT, AGXT2, SLC7A5, THmitochondrial matrix, extracellular space, cytosolOne of 5 routes

• 1-acylglycerone 3-phosphateReach from here →
  1. acetyl-CoA[m] + glyoxalate[m] + H2O[m] = CoA[m] + H+[m] + malate[m]
  2. acyl-CoA:sphingosine N-acyltransferase
  3. Acyl-CoA:glycerone-phosphate O-acyltransferase

  Catalysed by CLYBL, ASAH1, ACER2, ACER1 and 2 moremitochondrial matrix, Golgi apparatus, cytosol

• bromideReach from here →
  1. L-Alanine:glyoxylate aminotransferase
  2. gamma-L-glutamyl-L-cysteine:glycine ligase (ADP-forming)
  3. 2-bromoacetaldehyde:glutathione S-(formylmethyl)transferase

  Catalysed by AGXT, AGXT2, GSS, MGST3 and 16 moremitochondrial matrix, cytosolOne of 2 routes

• alpha-D-glucosamine 6-phosphateReach from here →
  1. L-Alanine:glyoxylate aminotransferase
  2. transport of glutamine and glycine (cytosol to extracellular)
  3. L-glutamine:D-fructose-6-phosphate isomerase (deaminating)

  Catalysed by AGXT, AGXT2, SLC7A5, GFPT2 and 1 moremitochondrial matrix, extracellular space, cytosol

• thioredoxin dithiolReach from here →
  1. L-Alanine:glyoxylate aminotransferase
  2. glycine:tRNA(Gly) ligase (AMP-forming)
  3. glycyl-tRNA(gly) phosphorylation

  Catalysed by AGXT, AGXT2, GARS1mitochondrial matrix, cytosolOne of 2 routes

• phosphatidylethanolamineReach from here →
  1. acetyl-CoA[m] + glyoxalate[m] + H2O[m] = CoA[m] + H+[m] + malate[m]
  2. acyl-CoA:sphingosine N-acyltransferase
  3. Acyl-CoA:1-acyl-sn-glycero-3-phosphoethanolamine O-acyltransferase

  Catalysed by CLYBL, ASAH1, ACER2, ACER1 and 6 moremitochondrial matrix, Golgi apparatus, cytosol

• a halide anionReach from here →
  1. L-Alanine:glyoxylate aminotransferase
  2. gamma-L-glutamyl-L-cysteine + glycine + ATP = glutathione + ADP + phosphate + H(+)
  3. RX + glutathione = an S-substituted glutathione + a halide anion + H(+)

  Catalysed by AGXT, AGXT2, GSS, GSTT4 and 22 moremitochondrial matrix

• 11-cis-retinalReach 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:NAD+ oxidoreductase

  Catalysed by CLYBL, DGAT1, LRAT, AWAT1 and 9 moremitochondrial matrix, cytosolOne of 4 routes

• 1,2-diacyl-sn-glycero-3-phosphocholine(1+)Reach from here →
  1. acetyl-CoA[m] + glyoxalate[m] + H2O[m] = CoA[m] + H+[m] + malate[m]
  2. acyl-CoA:sphingosine N-acyltransferase
  3. Acyl-CoA:1-acyl-sn-glycero-3-phosphocholine O-acyltransferase

  Catalysed by CLYBL, ASAH1, ACER2, ACER1 and 5 moremitochondrial matrix, Golgi apparatus, cytosol

• hexadecan-1-olReach from here →
  1. acetyl-CoA[m] + glyoxalate[m] + H2O[m] = CoA[m] + H+[m] + malate[m]
  2. Palmitate:CoA ligase (AMP-forming)
  3. formate dehydrogenase (palmitoyl-CoA)

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

• CHEBI:16174Reach from here →
  1. L-Alanine:glyoxylate aminotransferase
  2. pyruvate kinase (dTDP)
  3. nucleoside-diphosphate kinase (dADP)

  Catalysed by AGXT, AGXT2, PKM, PKLR and 6 moremitochondrial matrix, cytosolOne of 3 routes

• ureaReach from here →
  1. L-Alanine:glyoxylate aminotransferase
  2. transport of arginine and glycine (cytosol to extracellular)
  3. L-Arginine amidinohydrolase

  Catalysed by AGXT, AGXT2, SLC7A6, ARG1mitochondrial matrix, extracellular space, cytosolOne of 3 routes

• 11-cis-retinyl hexadecanoateReach from here →
  1. acetyl-CoA[m] + glyoxalate[m] + H2O[m] = CoA[m] + H+[m] + malate[m]
  2. Palmitate:CoA ligase (AMP-forming)
  3. Palmitoyl-CoA:retinol O-acyltransferase

  Catalysed by CLYBL, SLC27A2, ACSL4, ACSL3 and 10 moremitochondrial matrix, cytosolOne of 3 routes

• L-cystineReach from here →
  1. L-Alanine:glyoxylate aminotransferase
  2. transport of alanine and glycine (cytosol to extracellular)
  3. Transport of L-Cystine (L-Alanine antiport) by B0, +At Transporter

  Catalysed by AGXT, AGXT2, SLC3A2, SLC7A5 and 3 moremitochondrial matrix, extracellular space, cytosolOne of 8 routes

• CHEBI:16284Reach from here →
  1. L-Alanine:glyoxylate aminotransferase
  2. pyruvate kinase (dTDP)
  3. deoxythymidylate kinase (dADP)

  Catalysed by AGXT, AGXT2, PKM, PKLR and 1 moremitochondrial matrix, cytosolOne of 3 routes

• (R)-3-aminoisobutyric acidReach from here →
  1. L-Alanine:glyoxylate aminotransferase
  2. transport of alanine and glycine (cytosol to extracellular)
  3. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase

  Catalysed by AGXT, AGXT2, SLC3A2, SLC7A5 and 1 moremitochondrial matrix, extracellular space, cytosolOne of 3 routes

• lithocholic acidReach from here →
  1. L-Alanine:glyoxylate aminotransferase
  2. gamma-L-glutamyl-L-cysteine:glycine ligase (ADP-forming)
  3. transport of GSH and lithocholate (cytosol to extracellular)

  Catalysed by AGXT, AGXT2, GSS, SLCO1A2 and 1 moremitochondrial matrix, cytosol, extracellular spaceOne of 2 routes

• phosphatidic acidReach from here →
  1. acetyl-CoA[m] + glyoxalate[m] + H2O[m] = CoA[m] + H+[m] + malate[m]
  2. acyl-CoA:sphingosine N-acyltransferase
  3. acyl-CoA:1-acyl-sn-glycerol-3-phosphate 2-O-acyltransferase

  Catalysed by CLYBL, ASAH1, ACER2, ACER1 and 10 moremitochondrial matrix, Golgi apparatusOne of 7 routes

• iodideReach from here →
  1. L-Alanine:glyoxylate aminotransferase
  2. transport of chloride and glycine (cytosol to extracellular)
  3. transport of chloride and iodide (cytosol to extracellular)

  Catalysed by AGXT, AGXT2, SLC6A9, SLC6A14 and 1 moremitochondrial matrix, extracellular space, cytosolOne of 3 routes

• cis-aconitateReach from here →
  1. Glyoxylate:oxygen oxidoreductase
  2. Mitochondrial Carrier (Mc) Tcdb:2.A.29.7.2
  3. cis-aconitate(in) + citrate(out) = cis-aconitate(out) + citrate(in)

  Catalysed by HAO1, SLC25A1peroxisome, mitochondrial matrix, cytosol, inner side of the membrane, outer side of the membraneOne of 2 routes

• ubiquinonesReach from here →
  1. acetyl-CoA[m] + glyoxalate[m] + H2O[m] = CoA[m] + H+[m] + malate[m]
  2. (S)-malate hydro-lyase (fumarate-forming)
  3. succinate:quinone oxidoreductase

  Catalysed by CLYBL, FH, SDHD, SDHB and 2 moremitochondrial matrix, cytosol

• nitric oxideReach from here →
  1. L-Alanine:glyoxylate aminotransferase
  2. transport of arginine and glycine (cytosol to extracellular)
  3. L-Arginine, NADPH:Oxygen Oxidoreductase (Nitric-Oxide-Forming)

  Catalysed by AGXT, AGXT2, SLC7A6, NOS3 and 2 moremitochondrial matrix, extracellular space, cytosolOne of 2 routes

• CHEBI:16497Reach from here →
  1. L-Alanine:glyoxylate aminotransferase
  2. pyruvate kinase (dTDP)
  3. dGTP:pyruvate 2-O-phosphotransferase

  Catalysed by AGXT, AGXT2, PKM, PKLRmitochondrial matrix, cytosol

• N(2)-acetyl-L-ornithineReach from here →
  1. L-Alanine:glyoxylate aminotransferase
  2. L-Arginine:glycine amidinotransferase
  3. N2-Acetyl-L-ornithine amidohydrolase

  Catalysed by AGXT, AGXT2, GATM, ACY1mitochondrial matrix, cytosolOne of 2 routes

• 2-phenylacetamideReach from here →
  1. L-Alanine:glyoxylate aminotransferase
  2. transport of glycine and phenylalanine (cytosol to extracellular)
  3. L-phenylalanine:oxygen 2-oxidoreductase (decarboxylating)

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

• D-alanyl-D-alanineReach from here →
  1. L-Alanine:glyoxylate aminotransferase
  2. transport of D-alanine and glycine (cytosol to extracellular)
  3. Formation of Alanylalanine

  Catalysed by AGXT, AGXT2, SLC3A2, SLC7A10mitochondrial matrix, extracellular space, cytosol

• CHEBI:16633Reach from here →
  1. L-Alanine:glyoxylate aminotransferase
  2. transport of alanine and glycine (cytosol to extracellular)
  3. L-selenocysteine selenide-lyase (L-alanine-forming)

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

• Met-tRNA(Met)Reach from here →
  1. L-Alanine:glyoxylate aminotransferase
  2. transport of glycine and methionine (cytosol to extracellular)
  3. L-Methionine:tRNAMet ligase (AMP-forming)

  Catalysed by AGXT, AGXT2, SLC7A5, MARS1mitochondrial matrix, extracellular space, cytosol

• (R)-glycerateReach from here →
  1. L-Alanine:glyoxylate aminotransferase
  2. L-Serine:pyruvate aminotransferase
  3. D-glycerate:NADP+ 2-oxidoreductase

  Catalysed by AGXT, AGXT2, GRHPRmitochondrial matrix, cytosolOne of 4 routes

• quinolinic acidReach from here →
  1. Glyoxylate:oxygen oxidoreductase
  2. transport of oxalate and sulfate (cytosol to extracellular)
  3. Transport of Quinolinic Acid

  Catalysed by HAO1, SLC26A6, SLC22A6peroxisome, extracellular space, cytosolOne of 2 routes