4-methylsulfanyl-2-oxobutanoate

Three steps

• glycochenodeoxycholic acidReach from here →
  1. glyoxylate + L-methionine = 4-methylsulfanyl-2-oxobutanoate + glycine
  2. glycine + 2-oxoglutarate = glyoxylate + L-glutamate
  3. bile acid-CoA: amino acid N-acyltransferase

  Catalysed by AADAT, BAATperoxisomeOne of 3 routes

• 3-hydroxyisovaleric acidReach from here →
  1. glyoxylate + L-methionine = 4-methylsulfanyl-2-oxobutanoate + glycine
  2. L-leucine + glyoxylate = 4-methyl-2-oxopentanoate + glycine
  3. 4-methyl-2-oxopentanoate decarboxylation

  Catalysed by AADAT, ALOX12B, ALOXE3cytosol

• glycolithocholic acidReach from here →
  1. glyoxylate + L-methionine = 4-methylsulfanyl-2-oxobutanoate + glycine
  2. glycine + 2-oxoglutarate = glyoxylate + L-glutamate
  3. bile acid-CoA:amino acid N-acyltransferase (glycine)

  Catalysed by AADAT, BAATcytosolOne of 2 routes

• decanamideReach from here →
  1. glyoxylate + L-methionine = 4-methylsulfanyl-2-oxobutanoate + glycine
  2. N-decanoyl-(2S)-hydroxyglycine = decanamide + glyoxylate
  3. N-decanoyl-(2S)-hydroxyglycine = decanamide + glyoxylate

  Catalysed by AADAT, PAM

• L-2-amino-3-oxobutanoic acidReach from here →
  1. glyoxylate + L-methionine = 4-methylsulfanyl-2-oxobutanoate + glycine
  2. glycine + 2-oxoglutarate = glyoxylate + L-glutamate
  3. Acetyl-CoA:glycine C-acetyltransferase

  Catalysed by AADAT, GCATmitochondrial matrix

• (R)-lactic acidReach from here →
  1. glyoxylate + L-methionine = 4-methylsulfanyl-2-oxobutanoate + glycine
  2. glyoxylate + L-alanine = glycine + pyruvate
  3. (R)-Lactate:NAD+ oxidoreductase

  Catalysed by AADAT, AGXT, AGXT2, LDHDcytosol

• (S)-lactic acidReach from here →
  1. glyoxylate + L-methionine = 4-methylsulfanyl-2-oxobutanoate + glycine
  2. glyoxylate + L-alanine = glycine + pyruvate
  3. (S)-Lactate:NAD+ oxidoreductase

  Catalysed by AADAT, AGXT, AGXT2, LDHB and 1 moremitochondrial matrixOne of 7 routes

• 25-hydroxycholesterolReach 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. cholesterol + AH2 + O2 = 25-hydroxycholesterol + A + H2O

  Catalysed by AADAT, CDKAL1, CH25H

• L-erythro-7,8-dihydrobiopterinReach 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-erythro-7,8-dihydrobiopterin(out) + a dicarboxylate(in) = L-erythro-7,8-dihydrobiopterin(in) + a dicarboxylate(out)

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

• 2-iminopropanoateReach from here →
  1. glyoxylate + L-methionine = 4-methylsulfanyl-2-oxobutanoate + glycine
  2. glyoxylate + L-alanine = glycine + pyruvate
  3. 2-iminopropanoate + H2O = pyruvate + NH4(+)

  Catalysed by AADAT, AGXT, AGXT2, RIDA

• phosphoenolpyruvic acidReach 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

• N-acetyl-beta-neuraminic acidReach from here →
  1. glyoxylate + L-methionine = 4-methylsulfanyl-2-oxobutanoate + glycine
  2. glyoxylate + L-alanine = glycine + pyruvate
  3. N-Acetylneuraminate pyruvate-lyase (N-acetyl-D-mannosamine-forming)

  Catalysed by AADAT, AGXT, AGXT2, NPLcytosol

• gamma-Glu-AlaReach from here →
  1. glyoxylate + L-methionine = 4-methylsulfanyl-2-oxobutanoate + glycine
  2. transport of alanine and methionine (cytosol to extracellular)
  3. gamma-glutamyltransferase (alanine)

  Catalysed by AADAT, SLC7A5, GGT1, GGT5 and 4 moreextracellular space, cytosol

• S(8)-aminomethyldihydrolipoamideReach from here →
  1. glyoxylate + L-methionine = 4-methylsulfanyl-2-oxobutanoate + glycine
  2. glycine + 2-oxoglutarate = glyoxylate + L-glutamate
  3. dihydrolipoyl dehydrogenase ([protein]-N6-(lipoyl)lysine)

  Catalysed by AADAT, GLDC, GCSHmitochondrial matrix

• taurineReach from here →
  1. glyoxylate + L-methionine = 4-methylsulfanyl-2-oxobutanoate + glycine
  2. N-acetylglutamate synthase (N-acetylmethionine)
  3. bile acid-CoA:amino acid N-acyltransferase (taurodeoxycholate)

  Catalysed by AADAT, NAGS, BAATmitochondrial matrix, cytosolOne of 4 routes

• all-trans-13,14-dihydroretinolReach 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. all-trans-13,14-dihydroretinol + A = all-trans-retinol + AH2

  Catalysed by AADAT, CDKAL1, RETSAT

• 3,3',5-triiodo-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. 3,3',5-triiodo-L-thyronine + iodide + A + H(+) = L-thyroxine + AH2

  Catalysed by AADAT, CDKAL1, DIO1, DIO2

• 3,3',5'-triiodo-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. 3,3',5'-triiodo-L-thyronine + iodide + A + H(+) = L-thyroxine + AH2

  Catalysed by AADAT, CDKAL1, DIO1, DIO3

• a 3-acylpyruvateReach from here →
  1. glyoxylate + L-methionine = 4-methylsulfanyl-2-oxobutanoate + glycine
  2. glyoxylate + L-alanine = glycine + pyruvate
  3. a 3-acylpyruvate + H2O = a carboxylate + pyruvate + H(+)

  Catalysed by AADAT, AGXT, AGXT2, FAHD1

• (S)-malyl-CoAReach from here →
  1. glyoxylate + L-methionine = 4-methylsulfanyl-2-oxobutanoate + glycine
  2. glyoxylate + acetyl-CoA + H2O = (S)-malate + CoA + H(+)
  3. (S)-malate + ATP + CoA = (S)-malyl-CoA + ADP + phosphate

  Catalysed by AADAT, CLYBL, SUCLG1, SUCLA2

• (R)-methylmalonyl-CoAReach from here →
  1. glyoxylate + L-methionine = 4-methylsulfanyl-2-oxobutanoate + glycine
  2. N-acetylglutamate synthase (N-acetylmethionine)
  3. aldehyde dehydrogenase (2-methyl-3-oxopropanoate)

  Catalysed by AADAT, NAGS, ALDH6A1mitochondrial matrixOne of 2 routes

• (S)-methylmalonyl-CoAReach from here →
  1. glyoxylate + L-methionine = 4-methylsulfanyl-2-oxobutanoate + glycine
  2. N-acetylglutamate synthase (N-acetylmethionine)
  3. (S)-methylmalonyl-CoA hydrolase

  Catalysed by AADAT, NAGSmitochondrial matrix

• 3'-dephospho-CoAReach from here →
  1. glyoxylate + L-methionine = 4-methylsulfanyl-2-oxobutanoate + glycine
  2. N-acetylglutamate synthase (N-acetylmethionine)
  3. acid phosphatase 2, lysosomal (CoA)

  Catalysed by AADAT, NAGS, ACP2mitochondrial matrix, lysosomeOne of 2 routes

• (2E)-dodecenoyl-CoAReach from here →
  1. glyoxylate + L-methionine = 4-methylsulfanyl-2-oxobutanoate + glycine
  2. N-acetylglutamate synthase (N-acetylmethionine)
  3. (2E)-dodecenoyl-CoA formation

  Catalysed by AADAT, NAGSmitochondrial matrixOne of 3 routes

• 4,8,12-trimethyltridecanoyl-CoAReach from here →
  1. glyoxylate + L-methionine = 4-methylsulfanyl-2-oxobutanoate + glycine
  2. N-acetylglutamate synthase (N-acetylmethionine)
  3. propanoyl-CoA C-acyltransferase (3-ketopristanoyl-CoA)

  Catalysed by AADAT, NAGS, ACAA1, MYO5Bmitochondrial matrix, peroxisomeOne of 2 routes

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

  Catalysed by AADAT, NAGS, SLC27A2, ACSL4 and 6 moremitochondrial matrix, cytosolOne of 7 routes

• butanoyl-CoAReach from here →
  1. glyoxylate + L-methionine = 4-methylsulfanyl-2-oxobutanoate + glycine
  2. N-acetylglutamate synthase (N-acetylmethionine)
  3. Butanoate:CoA ligase (AMP-forming)

  Catalysed by AADAT, NAGS, ACSM4, ACSM1 and 4 moremitochondrial matrix, cytosolOne of 9 routes

• dodecanoyl-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 (lauric acid)

  Catalysed by AADAT, NAGS, SLC27A2, ACSL4 and 6 moremitochondrial matrix, cytosolOne of 9 routes

• glutaryl-CoAReach from here →
  1. glyoxylate + L-methionine = 4-methylsulfanyl-2-oxobutanoate + glycine
  2. N-acetylglutamate synthase (N-acetylmethionine)
  3. Production of Glutaryl Carnitine

  Catalysed by AADAT, NAGS, CPT1A, CPT1C and 1 moremitochondrial matrix, cytosolOne of 6 routes

• eicosanoyl-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 (eicosanoate)

  Catalysed by AADAT, NAGS, SLC27A2, ACSL4 and 6 moremitochondrial matrix, cytosolOne of 12 routes

• itaconyl-CoAReach from here →
  1. glyoxylate + L-methionine = 4-methylsulfanyl-2-oxobutanoate + glycine
  2. N-acetylglutamate synthase (N-acetylmethionine)
  3. Itaconate:CoA ligase (GDP-forming)

  Catalysed by AADAT, NAGS, SUCLG1, SUCLG2 and 1 moremitochondrial matrixOne of 2 routes

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

  Catalysed by AADAT, NAGS, SLC27A2, ACSL4 and 6 moremitochondrial matrix, cytosolOne of 13 routes

• tetradecanoyl-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 (myristic acid)

  Catalysed by AADAT, NAGS, SLC27A2, ACSL4 and 6 moremitochondrial matrix, cytosolOne of 11 routes

• octanoyl-CoAReach from here →
  1. glyoxylate + L-methionine = 4-methylsulfanyl-2-oxobutanoate + glycine
  2. N-acetylglutamate synthase (N-acetylmethionine)
  3. Octanoyl-CoA:L-carnitine O-octanoyltransferase

  Catalysed by AADAT, NAGS, CROTmitochondrial matrixOne of 20 routes

• phytanoyl-CoAReach from here →
  1. glyoxylate + L-methionine = 4-methylsulfanyl-2-oxobutanoate + glycine
  2. N-acetylglutamate synthase (N-acetylmethionine)
  3. phytanate-CoA ligase

  Catalysed by AADAT, NAGSmitochondrial matrix, cytosolOne of 5 routes

• propanoyl-CoAReach from here →
  1. glyoxylate + L-methionine = 4-methylsulfanyl-2-oxobutanoate + glycine
  2. propanoyl-CoA + glyoxylate + H2O = 3-methylmalate + CoA + H(+)
  3. propanoyl-CoA + glyoxylate + H2O = 3-methylmalate + CoA + H(+)

  Catalysed by AADAT, CLYBL

• octadecanoyl-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 (stearate)

  Catalysed by AADAT, NAGS, SLC27A2, ACSL4 and 5 moremitochondrial matrix, cytosolOne of 15 routes

• prostaglandin F2alphaReach from here →
  1. 4-methylsulfanyl-2-oxobutanoate + L-kynurenine = kynurenate + L-methionine + H2O
  2. kynurenate(out) + glutarate(in) = kynurenate(in) + glutarate(out)
  3. prostaglandin F2alpha(out) + glutarate(in) = prostaglandin F2alpha(in) + glutarate(out)

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

• prostaglandin H2Reach 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. (5Z,8Z,11Z,14Z)-eicosatetraenoate + AH2 + 2 O2 = prostaglandin H2 + A + H2O

  Catalysed by AADAT, CDKAL1, PTGS1, PTGS2One of 2 routes

• (15S)-hydroxy-(5Z,8Z,11Z,13E)-eicosatetraenoateReach 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. (15S)-hydroperoxy-(5Z,8Z,11Z,13E)-eicosatetraenoate + AH2 = (15S)-hydroxy-(5Z,8Z,11Z,13E)-eicosatetraenoate + A + H2O

  Catalysed by AADAT, CDKAL1, TBXAS1, PTGS2 and 1 more

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

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

• sarcosineReach from here →
  1. glyoxylate + L-methionine = 4-methylsulfanyl-2-oxobutanoate + glycine
  2. glycine + 2-oxoglutarate = glyoxylate + L-glutamate
  3. sarcosine + O2 + H2O = formaldehyde + glycine + H2O2

  Catalysed by AADAT, PIPOXOne of 2 routes

• (6S)-5,6,7,8-tetrahydrofolateReach from here →
  1. glyoxylate + L-methionine = 4-methylsulfanyl-2-oxobutanoate + glycine
  2. glycine + 2-oxoglutarate = glyoxylate + L-glutamate
  3. (6R)-5,10-methylene-5,6,7,8-tetrahydrofolate + glycine + H2O = (6S)-5,6,7,8-tetrahydrofolate + L-serine

  Catalysed by AADAT, SHMT1, SHMT2One of 2 routes

• 2-(N(omega)-L-arginino)succinateReach from here →
  1. glyoxylate + L-methionine = 4-methylsulfanyl-2-oxobutanoate + glycine
  2. transport of arginine and methionine (cytosol to extracellular)
  3. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)

  Catalysed by AADAT, SLC7A6, ASLextracellular space, cytosolOne of 6 routes

• 4-guanidinobutanoateReach from here →
  1. glyoxylate + L-methionine = 4-methylsulfanyl-2-oxobutanoate + glycine
  2. transport of arginine and methionine (cytosol to extracellular)
  3. 4-aminobutanoate + L-arginine = 4-guanidinobutanoate + L-ornithine

  Catalysed by AADAT, SLC7A6, GATMextracellular space, cytosol

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

  Catalysed by AADAT, SLC7A5, SLC7A8extracellular space, cytosol, inner side of the membrane, outer side of the membraneOne of 2 routes

• N(6)-(1,2-dicarboxyethyl)-AMPReach from here →
  1. glyoxylate + L-methionine = 4-methylsulfanyl-2-oxobutanoate + glycine
  2. L-aspartate(in) + L-methionine(out) = L-aspartate(out) + L-methionine(in)
  3. IMP:L-aspartate ligase (GDP-forming)

  Catalysed by AADAT, SLC7A13, ADSS2, ADSS1inner side of the membrane, outer side of the membrane, cytosolOne of 2 routes

• 3-guanidinopropanoateReach from here →
  1. glyoxylate + L-methionine = 4-methylsulfanyl-2-oxobutanoate + glycine
  2. transport of arginine and methionine (cytosol to extracellular)
  3. beta-alanine + L-arginine = 3-guanidinopropanoate + L-ornithine

  Catalysed by AADAT, SLC7A6, GATMextracellular space, cytosol

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

  Catalysed by AADAT, SLC7A5, SLC7A8extracellular space, cytosol, inner side of the membrane, outer side of the membraneOne of 7 routes

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

  Catalysed by AADAT, SLC7A6, SLC25A2extracellular space, cytosol, inner side of the membrane, outer side of the membraneOne of 2 routes