4-methylsulfanyl-2-oxobutanoate

Three steps

• trans-urocanateReach from here →
  1. glyoxylate + L-methionine = 4-methylsulfanyl-2-oxobutanoate + glycine
  2. transport of histidine and methionine (cytosol to extracellular)
  3. L-histidine ammonia-lyase (urocanate-forming)

  Catalysed by AADAT, SLC7A6, HALextracellular space, cytosol

• urateReach from here →
  1. 4-methylsulfanyl-2-oxobutanoate + L-kynurenine = kynurenate + L-methionine + H2O
  2. kynurenate(out) + glutarate(in) = kynurenate(in) + glutarate(out)
  3. urate(in) + glutarate(out) = urate(out) + glutarate(in)

  Catalysed by AADAT, SLC22A6, SLC22A8inner side of the membrane, outer side of the membraneOne of 4 routes

• 5-hydroxy-L-tryptophanReach from here →
  1. glyoxylate + L-methionine = 4-methylsulfanyl-2-oxobutanoate + glycine
  2. transport of methionine 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 AADAT, SLC7A5, TPH1, TPH2extracellular space, cytosol

• CHEBI:17927Reach from here →
  1. glyoxylate + L-methionine = 4-methylsulfanyl-2-oxobutanoate + glycine
  2. N-acetylglutamate synthase (N-acetylmethionine)
  3. N1,N8-diacetylspermidine to N1-acetylspermidine conversion

  Catalysed by AADAT, NAGSmitochondrial matrix, cytosolOne of 2 routes

• fumaric acidReach from here →
  1. glyoxylate + L-methionine = 4-methylsulfanyl-2-oxobutanoate + glycine
  2. glyoxylate + acetyl-CoA + H2O = (S)-malate + CoA + H(+)
  3. (S)-malate hydro-lyase (fumarate-forming)

  Catalysed by AADAT, CLYBL, FHcytosolOne of 5 routes

• dTDPReach from here →
  1. glyoxylate + L-methionine = 4-methylsulfanyl-2-oxobutanoate + glycine
  2. glyoxylate + L-alanine = glycine + pyruvate
  3. pyruvate kinase (dTDP)

  Catalysed by AADAT, AGXT, AGXT2, PKM and 1 morecytosol

• CHEBI:18095Reach from here →
  1. glyoxylate + L-methionine = 4-methylsulfanyl-2-oxobutanoate + glycine
  2. transport of alanine and methionine (cytosol to extracellular)
  3. Transport of Hydroxy-Proline

  Catalysed by AADAT, SLC7A5, SLC1A4extracellular space, cytosolOne of 5 routes

• N-carbamoyl-beta-alanineReach from here →
  1. glyoxylate + L-methionine = 4-methylsulfanyl-2-oxobutanoate + glycine
  2. transport of chloride and methionine (cytosol to extracellular)
  3. Transport of 3-Ureidopropionate

  Catalysed by AADAT, SLC6A14extracellular space, cytosolOne of 2 routes

• N(1)-(5-phospho-D-ribosyl)glycinamideReach from here →
  1. glyoxylate + L-methionine = 4-methylsulfanyl-2-oxobutanoate + glycine
  2. glycine + 2-oxoglutarate = glyoxylate + L-glutamate
  3. 5-Phospho-D-ribosylamine:glycine ligase (ADP-forming)

  Catalysed by AADAT, GARTcytosol

• 2-phenylethylamineReach from here →
  1. glyoxylate + L-methionine = 4-methylsulfanyl-2-oxobutanoate + glycine
  2. transport of methionine and phenylalanine (cytosol to extracellular)
  3. L-phenylalanine carboxy-lyase (phenylethylamine-forming)

  Catalysed by AADAT, SLC7A5, DDCextracellular space, cytosol

• 2-formamido-N(1)-(5-phospho-D-ribosyl)acetamidineReach from here →
  1. glyoxylate + L-methionine = 4-methylsulfanyl-2-oxobutanoate + glycine
  2. transport of glutamine and methionine (cytosol to extracellular)
  3. 5'-Phosphoribosylformylglycinamide:L-glutamine amido-ligase (ADP-forming)

  Catalysed by AADAT, SLC7A5, PFASextracellular space, cytosol

• ophthalmateReach from here →
  1. glyoxylate + L-methionine = 4-methylsulfanyl-2-oxobutanoate + glycine
  2. glycine + 2-oxoglutarate = glyoxylate + L-glutamate
  3. gamma-L-glutamyl-(2S)-2-aminobutanoate + glycine + ATP = ophthalmate + ADP + phosphate + H(+)

  Catalysed by AADAT, GSS

• 2-(alpha-Hydroxypropyl)thiamine diphosphateReach from here →
  1. glyoxylate + L-methionine = 4-methylsulfanyl-2-oxobutanoate + glycine
  2. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  3. branched chain keto acid dehydrogenase E1 (2-oxobutyrate)

  Catalysed by AADAT, AGXT2, BCKDHA, BCKDHBmitochondrial matrix

• 5alpha-androstane-3alpha,17beta-diol 3-O-(beta-D-glucuronate)Reach from here →
  1. 4-methylsulfanyl-2-oxobutanoate + L-kynurenine = kynurenate + L-methionine + H2O
  2. kynurenate(out) + glutarate(in) = kynurenate(in) + glutarate(out)
  3. 5alpha-androstane-3alpha,17beta-diol 3-O-(beta-D-glucuronate)(out) + glutarate(in) = 5alpha-androstane-3alpha,17beta-diol 3-O-(beta-D-glucuronate)(in) + glutarate(out)

  Catalysed by AADAT, SLC22A6, SLC22A24inner side of the membrane, outer side of the membraneOne of 2 routes

• 2-aminoisobutanoateReach from here →
  1. glyoxylate + L-methionine = 4-methylsulfanyl-2-oxobutanoate + glycine
  2. transport of alanine and methionine (cytosol to extracellular)
  3. 2-aminoisobutanoate(out) + L-alanine(in) = 2-aminoisobutanoate(in) + L-alanine(out)

  Catalysed by AADAT, SLC7A5, SLC7A10extracellular space, cytosol, inner side of the membrane, outer side of the membraneOne of 2 routes

• 3-carboxy-4-methyl-5-propyl-2-furanpropanoateReach from here →
  1. 4-methylsulfanyl-2-oxobutanoate + L-kynurenine = kynurenate + L-methionine + H2O
  2. kynurenate(out) + a dicarboxylate(in) = kynurenate(in) + a dicarboxylate(out)
  3. 3-carboxy-4-methyl-5-propyl-2-furanpropanoate(out) + a dicarboxylate(in) = 3-carboxy-4-methyl-5-propyl-2-furanpropanoate(in) + a dicarboxylate(out)

  Catalysed by AADAT, SLC22A8, SLC22A6inner side of the membrane, outer side of the membraneOne of 2 routes

• L-sepiapterinReach from here →
  1. 4-methylsulfanyl-2-oxobutanoate + L-kynurenine = kynurenate + L-methionine + H2O
  2. kynurenate(out) + a dicarboxylate(in) = kynurenate(in) + a dicarboxylate(out)
  3. L-sepiapterin(out) + a dicarboxylate(in) = L-sepiapterin(in) + a dicarboxylate(out)

  Catalysed by AADAT, SLC22A8, SLC22A6inner side of the membrane, outer side of the membraneOne of 2 routes

• (6R)-10-formyltetrahydrofolateReach from here →
  1. 1,2-dihydroxy-5-(methylsulfanyl)pent-1-en-3-one + O2 = 4-methylsulfanyl-2-oxobutanoate + formate + 2 H(+)
  2. 1,2-dihydroxy-5-(methylsulfanyl)pent-1-en-3-one + O2 = 3-(methylsulfanyl)propanoate + CO + formate + 2 H(+)
  3. (6S)-5,6,7,8-tetrahydrofolate + formate + ATP = (6R)-10-formyltetrahydrofolate + ADP + phosphate

  Catalysed by ADI1, MTHFD1, MTHFD1L

• 3',5'-diiodo-L-thyronineReach from here →
  1. glyoxylate + L-methionine = 4-methylsulfanyl-2-oxobutanoate + glycine
  2. N(6)-L-threonylcarbamoyladenosine(37) in tRNA + (sulfur carrier)-SH + AH2 + 2 S-adenosyl-L-methionine = 2-methylsulfanyl-N(6)-L-threonylcarbamoyladenosine(37) in tRNA + (sulfur carrier)-H + 5'-deoxyadenosine + L-methionine + A + S-adenosyl-L-homocysteine + 2 H(+)
  3. 3',5'-diiodo-L-thyronine + iodide + A + H(+) = 3,3',5'-triiodo-L-thyronine + AH2

  Catalysed by AADAT, CDKAL1, DIO3

• N(omega),N('omega)-dimethyl-L-arginineReach from here →
  1. glyoxylate + L-methionine = 4-methylsulfanyl-2-oxobutanoate + glycine
  2. glycine + 2-oxoglutarate = glyoxylate + L-glutamate
  3. N(omega),N('omega)-dimethyl-L-arginine + glyoxylate = 5-(3,3'-dimethylguanidino)-2-oxopentanoate + glycine

  Catalysed by AADAT, AGXT2One of 4 routes

• 5,6,7,8-tetrahydrofolic acidReach from here →
  1. glyoxylate + L-methionine = 4-methylsulfanyl-2-oxobutanoate + glycine
  2. glycine + 2-oxoglutarate = glyoxylate + L-glutamate
  3. 5,10-Methylenetetrahydrofolate:glycine hydroxymethyltransferase

  Catalysed by AADAT, SHMT1cytosolOne of 3 routes

• S-disulfanyl-L-cysteineReach from here →
  1. glyoxylate + L-methionine = 4-methylsulfanyl-2-oxobutanoate + glycine
  2. transport of cysteine and methionine (cytosol to extracellular)
  3. S-sulfanyl-L-cysteine + L-cysteine = S-disulfanyl-L-cysteine + L-alanine

  Catalysed by AADAT, SLC7A5, CARS2extracellular space, cytosol

• (5Z)-dodecenoyl-CoAReach from here →
  1. glyoxylate + L-methionine = 4-methylsulfanyl-2-oxobutanoate + glycine
  2. N-acetylglutamate synthase (N-acetylmethionine)
  3. acetyl-CoA C-acyltransferase (3-oxomyrist-7-enoyl-CoA)

  Catalysed by AADAT, NAGS, HADHA, ACAA2 and 1 moremitochondrial matrixOne of 2 routes

• 3-iodothyronamineReach from here →
  1. glyoxylate + L-methionine = 4-methylsulfanyl-2-oxobutanoate + glycine
  2. N(6)-L-threonylcarbamoyladenosine(37) in tRNA + (sulfur carrier)-SH + AH2 + 2 S-adenosyl-L-methionine = 2-methylsulfanyl-N(6)-L-threonylcarbamoyladenosine(37) in tRNA + (sulfur carrier)-H + 5'-deoxyadenosine + L-methionine + A + S-adenosyl-L-homocysteine + 2 H(+)
  3. 3-iodothyronamine + iodide + A + H(+) = 3,5-diiodothyronamine + AH2

  Catalysed by AADAT, CDKAL1, DIO1, DIO3One of 2 routes

• (11Z)-docosenoyl-CoA(4-)Reach from here →
  1. glyoxylate + L-methionine = 4-methylsulfanyl-2-oxobutanoate + glycine
  2. N-acetylglutamate synthase (N-acetylmethionine)
  3. long-chain-fatty-acid-CoA ligase (cis-cetoleic acid)

  Catalysed by AADAT, NAGS, SLC27A2, ACSL4 and 6 moremitochondrial matrix, cytosolOne of 5 routes

• 3-iodo-L-thyronineReach from here →
  1. glyoxylate + L-methionine = 4-methylsulfanyl-2-oxobutanoate + glycine
  2. N(6)-L-threonylcarbamoyladenosine(37) in tRNA + (sulfur carrier)-SH + AH2 + 2 S-adenosyl-L-methionine = 2-methylsulfanyl-N(6)-L-threonylcarbamoyladenosine(37) in tRNA + (sulfur carrier)-H + 5'-deoxyadenosine + L-methionine + A + S-adenosyl-L-homocysteine + 2 H(+)
  3. 3-iodo-L-thyronine + iodide + A + H(+) = 3,5-diiodo-L-thyronine + AH2

  Catalysed by AADAT, CDKAL1, DIO1, DIO3One of 2 routes

• (7Z)-tetradecenoyl-CoAReach from here →
  1. glyoxylate + L-methionine = 4-methylsulfanyl-2-oxobutanoate + glycine
  2. N-acetylglutamate synthase (N-acetylmethionine)
  3. long-chain-fatty-acid-CoA ligase ((7Z)-tetradecenoic acid)

  Catalysed by AADAT, NAGS, SLC27A2, ACSL4 and 6 moremitochondrial matrix, cytosolOne of 8 routes

• 3'-iodo-L-thyronineReach from here →
  1. glyoxylate + L-methionine = 4-methylsulfanyl-2-oxobutanoate + glycine
  2. N(6)-L-threonylcarbamoyladenosine(37) in tRNA + (sulfur carrier)-SH + AH2 + 2 S-adenosyl-L-methionine = 2-methylsulfanyl-N(6)-L-threonylcarbamoyladenosine(37) in tRNA + (sulfur carrier)-H + 5'-deoxyadenosine + L-methionine + A + S-adenosyl-L-homocysteine + 2 H(+)
  3. 3'-iodo-L-thyronine + iodide + A + H(+) = 3',5'-diiodo-L-thyronine + AH2

  Catalysed by AADAT, CDKAL1, DIO1, DIO2One of 2 routes

• L-thyronineReach from here →
  1. glyoxylate + L-methionine = 4-methylsulfanyl-2-oxobutanoate + glycine
  2. N(6)-L-threonylcarbamoyladenosine(37) in tRNA + (sulfur carrier)-SH + AH2 + 2 S-adenosyl-L-methionine = 2-methylsulfanyl-N(6)-L-threonylcarbamoyladenosine(37) in tRNA + (sulfur carrier)-H + 5'-deoxyadenosine + L-methionine + A + S-adenosyl-L-homocysteine + 2 H(+)
  3. L-thyronine + iodide + A + H(+) = 3-iodo-L-thyronine + AH2

  Catalysed by AADAT, CDKAL1, DIO3

• thyronamineReach from here →
  1. glyoxylate + L-methionine = 4-methylsulfanyl-2-oxobutanoate + glycine
  2. N(6)-L-threonylcarbamoyladenosine(37) in tRNA + (sulfur carrier)-SH + AH2 + 2 S-adenosyl-L-methionine = 2-methylsulfanyl-N(6)-L-threonylcarbamoyladenosine(37) in tRNA + (sulfur carrier)-H + 5'-deoxyadenosine + L-methionine + A + S-adenosyl-L-homocysteine + 2 H(+)
  3. thyronamine + iodide + A + H(+) = 3-iodothyronamine + AH2

  Catalysed by AADAT, CDKAL1, DIO3

• 3'-iodothyronamineReach from here →
  1. glyoxylate + L-methionine = 4-methylsulfanyl-2-oxobutanoate + glycine
  2. N(6)-L-threonylcarbamoyladenosine(37) in tRNA + (sulfur carrier)-SH + AH2 + 2 S-adenosyl-L-methionine = 2-methylsulfanyl-N(6)-L-threonylcarbamoyladenosine(37) in tRNA + (sulfur carrier)-H + 5'-deoxyadenosine + L-methionine + A + S-adenosyl-L-homocysteine + 2 H(+)
  3. 3'-iodothyronamine + iodide + A + H(+) = 3',5'-diiodothyronamine + AH2

  Catalysed by AADAT, CDKAL1, DIO1, DIO2One of 2 routes

• 3,3'-diiodothyronamineReach from here →
  1. glyoxylate + L-methionine = 4-methylsulfanyl-2-oxobutanoate + glycine
  2. N(6)-L-threonylcarbamoyladenosine(37) in tRNA + (sulfur carrier)-SH + AH2 + 2 S-adenosyl-L-methionine = 2-methylsulfanyl-N(6)-L-threonylcarbamoyladenosine(37) in tRNA + (sulfur carrier)-H + 5'-deoxyadenosine + L-methionine + A + S-adenosyl-L-homocysteine + 2 H(+)
  3. 3,3'-diiodothyronamine + iodide + A + H(+) = 3,3',5'-triiodothyronamine + AH2

  Catalysed by AADAT, CDKAL1, DIO1, DIO2One of 2 routes

• 3',5'-diiodothyronamineReach from here →
  1. glyoxylate + L-methionine = 4-methylsulfanyl-2-oxobutanoate + glycine
  2. N(6)-L-threonylcarbamoyladenosine(37) in tRNA + (sulfur carrier)-SH + AH2 + 2 S-adenosyl-L-methionine = 2-methylsulfanyl-N(6)-L-threonylcarbamoyladenosine(37) in tRNA + (sulfur carrier)-H + 5'-deoxyadenosine + L-methionine + A + S-adenosyl-L-homocysteine + 2 H(+)
  3. 3',5'-diiodothyronamine + iodide + A + H(+) = 3,3',5'-triiodothyronamine + AH2

  Catalysed by AADAT, CDKAL1, DIO3

• 3,3',5'-triiodothyronamineReach from here →
  1. glyoxylate + L-methionine = 4-methylsulfanyl-2-oxobutanoate + glycine
  2. N(6)-L-threonylcarbamoyladenosine(37) in tRNA + (sulfur carrier)-SH + AH2 + 2 S-adenosyl-L-methionine = 2-methylsulfanyl-N(6)-L-threonylcarbamoyladenosine(37) in tRNA + (sulfur carrier)-H + 5'-deoxyadenosine + L-methionine + A + S-adenosyl-L-homocysteine + 2 H(+)
  3. 3,3',5'-triiodothyronamine + iodide + A + H(+) = 3,3',5,5'-tetraiodothyronamine + AH2

  Catalysed by AADAT, CDKAL1, DIO3

• L-homocysteine thiolactoneReach from here →
  1. glyoxylate + L-methionine = 4-methylsulfanyl-2-oxobutanoate + glycine
  2. S-methyl-L-methionine + L-homocysteine = 2 L-methionine + H(+)
  3. L-homocysteine + ATP = L-homocysteine thiolactone + AMP + diphosphate

  Catalysed by AADAT, BHMT2, MARS1

• L-homocysteinyl-AMPReach from here →
  1. glyoxylate + L-methionine = 4-methylsulfanyl-2-oxobutanoate + glycine
  2. S-methyl-L-methionine + L-homocysteine = 2 L-methionine + H(+)
  3. L-homocysteine + ATP + H(+) = L-homocysteinyl-AMP + diphosphate

  Catalysed by AADAT, BHMT2, MARS1

• (4Z,7Z,10Z,13Z)-hexadecatetraenoyl-CoAReach from here →
  1. glyoxylate + L-methionine = 4-methylsulfanyl-2-oxobutanoate + glycine
  2. N-acetylglutamate synthase (N-acetylmethionine)
  3. Fatty Acid Beta Oxidation (C18:4->C16:4), Mitochondrial

  Catalysed by AADAT, NAGS, HADHA, ACADVL and 1 moremitochondrial matrixOne of 3 routes

• (7Z,10Z)-hexadecadienoyl-CoAReach from here →
  1. glyoxylate + L-methionine = 4-methylsulfanyl-2-oxobutanoate + glycine
  2. N-acetylglutamate synthase (N-acetylmethionine)
  3. Fatty Acid Beta Oxidation Trans (C18:2->C16:2), Mitochondrial

  Catalysed by AADAT, NAGS, HADHA, ACADVL and 1 moremitochondrial matrix

• (4Z,7Z)-decadienoyl-CoAReach from here →
  1. glyoxylate + L-methionine = 4-methylsulfanyl-2-oxobutanoate + glycine
  2. N-acetylglutamate synthase (N-acetylmethionine)
  3. Fatty Acid Beta Oxidation (C12:3->C10:2), Mitochondrial

  Catalysed by AADAT, NAGS, ECHS1, ACAA2 and 1 moremitochondrial matrixOne of 2 routes

• (5Z)-octenoyl-CoAReach from here →
  1. glyoxylate + L-methionine = 4-methylsulfanyl-2-oxobutanoate + glycine
  2. N-acetylglutamate synthase (N-acetylmethionine)
  3. Fatty Acid Beta Oxidation (C10:2->C8:1), Mitochondrial

  Catalysed by AADAT, NAGS, ECHS1, ACAA2 and 1 moremitochondrial matrixOne of 2 routes

• CHEBI:240943Reach from here →
  1. glyoxylate + L-methionine = 4-methylsulfanyl-2-oxobutanoate + glycine
  2. glycine + 2-oxoglutarate = glyoxylate + L-glutamate
  3. glycine N-acyltransferase (2-methylbutyryl-CoA)

  Catalysed by AADAT, GLYAT, GLYATL2, GLYATL1mitochondrial matrixOne of 3 routes

• a quinolReach from here →
  1. glyoxylate + L-methionine = 4-methylsulfanyl-2-oxobutanoate + glycine
  2. glyoxylate + acetyl-CoA + H2O = (S)-malate + CoA + H(+)
  3. (S)-malate + a quinone = enol-oxaloacetate + a quinol

  Catalysed by AADAT, CLYBL, SDHB, SDHA

• hypochlorous acidReach from here →
  1. glyoxylate + L-methionine = 4-methylsulfanyl-2-oxobutanoate + glycine
  2. transport of chloride and methionine (cytosol to extracellular)
  3. Peroxidase

  Catalysed by AADAT, SLC6A14, LPOextracellular space, cytosolOne of 4 routes

• potassium atomReach from here →
  1. glyoxylate + L-methionine = 4-methylsulfanyl-2-oxobutanoate + glycine
  2. transport of chloride and methionine (cytosol to extracellular)
  3. transport of chloride (cytosol to extracellular)

  Catalysed by AADAT, SLC6A14, SLC12A2, SLC12A1extracellular space, cytosolOne of 3 routes

• phenylacetylglycineReach from here →
  1. glyoxylate + L-methionine = 4-methylsulfanyl-2-oxobutanoate + glycine
  2. glycine + 2-oxoglutarate = glyoxylate + L-glutamate
  3. phenylacetyl-CoA:glycine N-phenylacetyltransferase

  Catalysed by AADAT, GLYATcytosolOne of 3 routes

• CHEBI:27540Reach from here →
  1. glyoxylate + L-methionine = 4-methylsulfanyl-2-oxobutanoate + glycine
  2. N-acetylglutamate synthase (N-acetylmethionine)
  3. medium-chain acyl-CoA ligase (hexanoic acid)

  Catalysed by AADAT, NAGS, ACSM4, ACSM1 and 4 moremitochondrial matrix, cytosolOne of 6 routes

• selenomethionineReach from here →
  1. glyoxylate + L-methionine = 4-methylsulfanyl-2-oxobutanoate + glycine
  2. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  3. Selenomethionine methanethiol-lyase (deaminating)

  Catalysed by AADAT, AGXT2, CTHcytosol

• CHEBI:27760Reach from here →
  1. glyoxylate + L-methionine = 4-methylsulfanyl-2-oxobutanoate + glycine
  2. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  3. Selenocystathionine Lysteine-lyase (deaminating)

  Catalysed by AADAT, AGXT2, CTHcytosol

• elaidic acidReach from here →
  1. glyoxylate + L-methionine = 4-methylsulfanyl-2-oxobutanoate + glycine
  2. N-acetylglutamate synthase (N-acetylmethionine)
  3. long-chain-fatty-acid-CoA ligase (elaidate)

  Catalysed by AADAT, NAGS, SLC27A2, ACSL4 and 6 moremitochondrial matrix, cytosolOne of 5 routes

• 5-hydroxytryptophanReach from here →
  1. glyoxylate + L-methionine = 4-methylsulfanyl-2-oxobutanoate + glycine
  2. transport of methionine and tryptophan (cytosol to extracellular)
  3. L-Tryptophan, Tetrahydrobiopterin:Oxygen Oxidoreductase (5-Hydroxylating)

  Catalysed by AADAT, SLC7A5, TPH1, TPH2extracellular space, cytosolOne of 2 routes