4-methylsulfanyl-2-oxobutanoate

Three steps

• an N-acylglycineReach from here →
  1. glyoxylate + L-methionine = 4-methylsulfanyl-2-oxobutanoate + glycine
  2. glycine + 2-oxoglutarate = glyoxylate + L-glutamate
  3. an acyl-CoA + glycine = an N-acylglycine + CoA + H(+)

  Catalysed by AADAT, GLYATL3, GLYAT, GLYATL2

• 2-oxo-3-sulfanylpropanoateReach from here →
  1. glyoxylate + L-methionine = 4-methylsulfanyl-2-oxobutanoate + glycine
  2. glyoxylate + L-alanine = glycine + pyruvate
  3. 2-oxo-3-sulfanylpropanoate + [thioredoxin]-dithiol = [thioredoxin]-disulfide + hydrogen sulfide + pyruvate + H(+)

  Catalysed by AADAT, AGXT, AGXT2, MPSTOne of 4 routes

• S-formylglutathioneReach 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. S-formylglutathione + H2O = formate + glutathione + H(+)

  Catalysed by ADI1, ESD

• quercetinReach from here →
  1. 1,2-dihydroxy-5-(methylsulfanyl)pent-1-en-3-one + O2 = 4-methylsulfanyl-2-oxobutanoate + formate + 2 H(+)
  2. acireductone dioxygenase
  3. quercetin + O2 = 2-(3,4-dihydroxybenzoyloxy)-4,6-dihydroxybenzoate + CO

  Catalysed by ADI1, PIRcytosol

• 2-methyl-3-oxopropanoateReach from here →
  1. glyoxylate + L-methionine = 4-methylsulfanyl-2-oxobutanoate + glycine
  2. glyoxylate + L-alanine = glycine + pyruvate
  3. (R)-3-amino-2-methylpropanoate + pyruvate = 2-methyl-3-oxopropanoate + L-alanine

  Catalysed by AADAT, AGXT, AGXT2

• guanidinoacetateReach from here →
  1. glyoxylate + L-methionine = 4-methylsulfanyl-2-oxobutanoate + glycine
  2. glycine + 2-oxoglutarate = glyoxylate + L-glutamate
  3. L-Arginine:glycine amidinotransferase

  Catalysed by AADAT, GATMcytosolOne of 5 routes

• 3',5'-cyclic GMPReach 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',5'-cyclic GMP(out) + a dicarboxylate(in) = 3',5'-cyclic GMP(in) + a dicarboxylate(out)

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

• sphing-4-enineReach from here →
  1. glyoxylate + L-methionine = 4-methylsulfanyl-2-oxobutanoate + glycine
  2. N-acetylglutamate synthase (N-acetylmethionine)
  3. acyl-CoA:sphingosine N-acyltransferase

  Catalysed by AADAT, NAGS, ASAH1, ACER2 and 2 moremitochondrial matrix, Golgi apparatusOne of 2 routes

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

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

• spermidineReach from here →
  1. glyoxylate + L-methionine = 4-methylsulfanyl-2-oxobutanoate + glycine
  2. N-acetylglutamate synthase (N-acetylmethionine)
  3. diamine N-acetyltransferase (N1-acetylspermidine)

  Catalysed by AADAT, NAGS, SAT1, SAT2mitochondrial matrix, cytosolOne of 3 routes

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

  Catalysed by AADAT, SLC22A6, SLC22A24, SLC22A8inner side of the membrane, outer side of the membraneOne of 2 routes

• L-dopaquinoneReach from here →
  1. glyoxylate + L-methionine = 4-methylsulfanyl-2-oxobutanoate + glycine
  2. transport of methionine and tyrosine (cytosol to extracellular)
  3. L-Tyrosine,L-dopa:oxygen oxidoreductase

  Catalysed by AADAT, SLC7A5, TYRextracellular space, cytosol

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

  Catalysed by AADAT, GSS

• L-threonineReach from here →
  1. glyoxylate + L-methionine = 4-methylsulfanyl-2-oxobutanoate + glycine
  2. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine
  3. L-threonine = 2-oxobutanoate + NH4(+)

  Catalysed by AADAT, AGXT2, SDS, SDSLOne of 9 routes

• L-saccharopineReach from here →
  1. glyoxylate + L-methionine = 4-methylsulfanyl-2-oxobutanoate + glycine
  2. transport of lysine and methionine (cytosol to extracellular)
  3. N6-(L-1,3-Dicarboxypropyl)-L-lysine:NADP+ oxidoreductase (L-lysine-forming)

  Catalysed by AADAT, SLC7A6, AASSextracellular space, cytosol, mitochondrial matrix

• L-kynurenineReach from here →
  1. glyoxylate + L-methionine = 4-methylsulfanyl-2-oxobutanoate + glycine
  2. transport of alanine and methionine (cytosol to extracellular)
  3. L-kynurenine + H2O = anthranilate + L-alanine + H(+)

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

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

  Catalysed by AADAT, SLC7A5, SLC7A10extracellular space, cytosol, inner side of the membrane, outer side of the membraneOne of 6 routes

• leukotriene C4Reach from here →
  1. glyoxylate + L-methionine = 4-methylsulfanyl-2-oxobutanoate + glycine
  2. glycine + 2-oxoglutarate = glyoxylate + L-glutamate
  3. leukotriene C4 hydrolysis

  Catalysed by AADATcytosol

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

  Catalysed by AADAT, SLC7A6, GATMextracellular space, cytosol

• L-glutamate 5-semialdehydeReach from here →
  1. glyoxylate + L-methionine = 4-methylsulfanyl-2-oxobutanoate + glycine
  2. transport of methionine and ornithine (cytosol to extracellular)
  3. L-ornithine + 2-oxoglutarate = L-glutamate 5-semialdehyde + L-glutamate

  Catalysed by AADAT, SLC7A6, OATextracellular space, cytosol

• N(4)-(beta-N-acetyl-D-glucosaminyl)-L-asparagineReach 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. N(4)-(beta-N-acetyl-D-glucosaminyl)-L-asparagine + H2O = N-acetyl-beta-D-glucosaminylamine + L-aspartate + H(+)

  Catalysed by AADAT, SLC7A13, AGAinner side of the membrane, outer side of the membrane

• 3-hydroxy-L-kynurenineReach from here →
  1. glyoxylate + L-methionine = 4-methylsulfanyl-2-oxobutanoate + glycine
  2. transport of alanine and methionine (cytosol to extracellular)
  3. 3-hydroxy-L-kynurenine + H2O = 3-hydroxyanthranilate + L-alanine + H(+)

  Catalysed by AADAT, SLC7A5, KYNUextracellular space, cytosol

• L,L-cystathionineReach from here →
  1. glyoxylate + L-methionine = 4-methylsulfanyl-2-oxobutanoate + glycine
  2. N-acetylglutamate synthase (N-acetylmethionine)
  3. N-acetylglutamate synthase (N-acetyl-L-cystathionine)

  Catalysed by AADAT, NAGSmitochondrial matrixOne of 2 routes

• 3',5'-cyclic AMPReach 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',5'-cyclic AMP(out) + a dicarboxylate(in) = 3',5'-cyclic AMP(in) + a dicarboxylate(out)

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

• gamma-L-glutamyl-L-cysteineReach from here →
  1. glyoxylate + L-methionine = 4-methylsulfanyl-2-oxobutanoate + glycine
  2. transport of cysteine and methionine (cytosol to extracellular)
  3. L-glutamate:L-cysteine gamma-ligase (ADP-forming)

  Catalysed by AADAT, SLC7A5, GCLC, GCLMextracellular space, cytosolOne of 2 routes

• 6-amino-2-oxohexanoateReach from here →
  1. glyoxylate + L-methionine = 4-methylsulfanyl-2-oxobutanoate + glycine
  2. transport of lysine and methionine (cytosol to extracellular)
  3. L-lysine:oxygen 2-oxidoreductase (deaminating)

  Catalysed by AADAT, SLC7A6extracellular space, cytosol, peroxisome

• carbamoyl phosphateReach from here →
  1. glyoxylate + L-methionine = 4-methylsulfanyl-2-oxobutanoate + glycine
  2. transport of glutamine and methionine (cytosol to extracellular)
  3. HCO3-:L-glutamine amido-ligase (ADP-forming, carbamate-phosphorylating)

  Catalysed by AADAT, SLC7A5, CADextracellular space, cytosolOne of 4 routes

• N,N-dimethylglycineReach 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 + glycine betaine = N,N-dimethylglycine + L-methionine

  Catalysed by AADAT, BHMT2, BHMT

• L-tyrosineReach from here →
  1. glyoxylate + L-methionine = 4-methylsulfanyl-2-oxobutanoate + glycine
  2. glycine + 2-oxoglutarate = glyoxylate + L-glutamate
  3. L-tyrosine + glyoxylate = 3-(4-hydroxyphenyl)pyruvate + glycine

  Catalysed by AADATOne of 6 routes

• 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 13 routes

• an acyl-CoAReach from here →
  1. glyoxylate + L-methionine = 4-methylsulfanyl-2-oxobutanoate + glycine
  2. N-acetylglutamate synthase (N-acetylmethionine)
  3. acyl-CoA:sphingosine N-acyltransferase

  Catalysed by AADAT, NAGS, ASAH1, ACER2 and 2 moremitochondrial matrix, Golgi apparatusOne of 2 routes

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

  Catalysed by AADAT, SLC7A5, SLC1A4extracellular space, cytosol, inner side of the membrane, outer side of the membraneOne of 2 routes

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

  Catalysed by AADAT, SLC7A6, HDCextracellular space, cytosol

• (2S)-2-[5-amino-1-(5-phospho-beta-D-ribosyl)imidazole-4-carboxamido]succinateReach 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. 1-(5-Phosphoribosyl)-5-amino-4-carboxyimidazole:L-aspartate ligase (ADP-forming)

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

• 5-guanidino-2-oxopentanoateReach from here →
  1. glyoxylate + L-methionine = 4-methylsulfanyl-2-oxobutanoate + glycine
  2. transport of arginine and methionine (cytosol to extracellular)
  3. L-arginine + O2 + H2O = 5-guanidino-2-oxopentanoate + H2O2 + NH4(+)

  Catalysed by AADAT, SLC7A6, IL4I1extracellular space, cytosol

• (3Z)-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-5-enoyl-CoA)

  Catalysed by AADAT, NAGS, HADHA, ACAA2 and 1 moremitochondrial matrixOne of 3 routes

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

  Catalysed by AADAT, SLC7A5, CARS2extracellular space, cytosol

• N-formyl-L-kynurenineReach from here →
  1. glyoxylate + L-methionine = 4-methylsulfanyl-2-oxobutanoate + glycine
  2. transport of methionine and tryptophan (cytosol to extracellular)
  3. L-tryptophan + O2 = N-formyl-L-kynurenine

  Catalysed by AADAT, SLC7A5, IDO1, TDO2 and 1 moreextracellular space, cytosolOne of 2 routes

• (3S)-citramalyl-CoAReach from here →
  1. glyoxylate + L-methionine = 4-methylsulfanyl-2-oxobutanoate + glycine
  2. glyoxylate + L-alanine = glycine + pyruvate
  3. (3S)-citramalyl-CoA = pyruvate + acetyl-CoA

  Catalysed by AADAT, AGXT, AGXT2, CLYBL

• L-2-aminoadipateReach from here →
  1. glyoxylate + L-methionine = 4-methylsulfanyl-2-oxobutanoate + glycine
  2. L-2-aminoadipate + glyoxylate = 2-oxoadipate + glycine
  3. 2-oxoadipate + L-kynurenine = L-2-aminoadipate + kynurenate + H2O

  Catalysed by AADAT

• (25R)-3alpha,7alpha,12alpha-trihydroxy-5beta-cholestan-26-oyl-CoAReach from here →
  1. glyoxylate + L-methionine = 4-methylsulfanyl-2-oxobutanoate + glycine
  2. N-acetylglutamate synthase (N-acetylmethionine)
  3. 3alpha,7alpha,12alpha-trihydroxy-5beta-cholestanoate:CoA ligase (AMP-forming)

  Catalysed by AADAT, NAGS, SLC27A2, SLC27A5mitochondrial matrix, endoplasmic reticulumOne of 3 routes

• 5-phospho-beta-D-ribosylamineReach from here →
  1. glyoxylate + L-methionine = 4-methylsulfanyl-2-oxobutanoate + glycine
  2. transport of glutamine and methionine (cytosol to extracellular)
  3. phosphoribosyl pyrophosphate amidotransferase (glutamine)

  Catalysed by AADAT, SLC7A5, PPATextracellular space, cytosolOne of 2 routes

• D-glucosamine 6-phosphateReach from here →
  1. glyoxylate + L-methionine = 4-methylsulfanyl-2-oxobutanoate + glycine
  2. transport of glutamine and methionine (cytosol to extracellular)
  3. D-fructose 6-phosphate + L-glutamine = D-glucosamine 6-phosphate + L-glutamate

  Catalysed by AADAT, SLC7A5, GFPT2, GFPT1extracellular space, cytosol

• deoxycholoyl-CoAReach from here →
  1. glyoxylate + L-methionine = 4-methylsulfanyl-2-oxobutanoate + glycine
  2. N-acetylglutamate synthase (N-acetylmethionine)
  3. cholate-CoA ligase (3alpha,12alpha-dihydroxy-5beta-cholanate)

  Catalysed by AADAT, NAGS, SLC27A2, SLC27A5mitochondrial matrix, cytosolOne of 4 routes

• N-(5Z,8Z,11Z,14Z)-eicosatetraenoyl-glycineReach from here →
  1. glyoxylate + L-methionine = 4-methylsulfanyl-2-oxobutanoate + glycine
  2. glycine + 2-oxoglutarate = glyoxylate + L-glutamate
  3. (5Z,8Z,11Z,14Z)-eicosatetraenoyl-CoA + glycine = N-(5Z,8Z,11Z,14Z)-eicosatetraenoyl-glycine + CoA + H(+)

  Catalysed by AADAT, GLYATL2One of 2 routes

• N-[(R)-4-phosphopantothenoyl]-L-cysteineReach from here →
  1. glyoxylate + L-methionine = 4-methylsulfanyl-2-oxobutanoate + glycine
  2. transport of cysteine and methionine (cytosol to extracellular)
  3. (R)-4'-phosphopantothenate:L-cysteine ligase

  Catalysed by AADAT, SLC7A5, PPCSextracellular space, cytosolOne of 4 routes

• (6R)-L-erythro-5,6,7,8-tetrahydrobiopterinReach 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. (6R)-L-erythro-5,6,7,8-tetrahydrobiopterin(out) + a dicarboxylate(in) = (6R)-L-erythro-5,6,7,8-tetrahydrobiopterin(in) + a dicarboxylate(out)

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

• 2-hydroxy-4-methylvaleric 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 reduction

  Catalysed by AADAT, SDR42E2, AKR1B15, AKR1B10 and 15 morecytosol

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

  Catalysed by AADAT, SLC6A14, SLC6A12extracellular space, cytosolOne of 4 routes

• 3-iodo-L-tyrosineReach from here →
  1. glyoxylate + L-methionine = 4-methylsulfanyl-2-oxobutanoate + glycine
  2. transport of methionine and tyrosine (cytosol to extracellular)
  3. thyroid peroxidase (iodine)

  Catalysed by AADAT, SLC7A5, TPOextracellular space, cytosolOne of 2 routes