A

Three steps

• eicosanoyl-CoAReach from here →
  1. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  2. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  3. long-chain-fatty-acid-CoA ligase (eicosanoate)

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

• itaconyl-CoAReach from here →
  1. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  2. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  3. Itaconate:CoA ligase (GDP-forming)

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

• (9Z,12Z)-octadecadienoyl-CoAReach from here →
  1. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  2. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  3. long-chain-fatty-acid-CoA ligase ((6Z,9Z)-octadecadienoic acid)

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

• tetradecanoyl-CoAReach from here →
  1. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  2. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  3. long-chain-fatty-acid-CoA ligase (myristic acid)

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

• octanoyl-CoAReach from here →
  1. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  2. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  3. Octanoyl-CoA:L-carnitine O-octanoyltransferase

  Catalysed by MOCOS, NAGS, CROTmitochondrial matrixOne of 20 routes

• phytanoyl-CoAReach from here →
  1. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  2. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  3. phytanate-CoA ligase

  Catalysed by MOCOS, NAGSmitochondrial matrix, cytosolOne of 5 routes

• octadecanoyl-CoAReach from here →
  1. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  2. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  3. long-chain-fatty-acid-CoA ligase (stearate)

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

• prostaglandin E1Reach from here →
  1. A + NADH + H(+) = AH2 + NAD(+)
  2. prostaglandin G1 + AH2 = prostaglandin H1 + A + H2O
  3. prostaglandin-E synthase (prostaglandin E1)

  Catalysed by AIFM1, PYROXD1, PTGS2, PTGES and 2 morecytosolOne of 4 routes

• prostaglandin I2Reach from here →
  1. A + NADH + H(+) = AH2 + NAD(+)
  2. (5Z,8Z,11Z,14Z)-eicosatetraenoate + AH2 + 2 O2 = prostaglandin H2 + A + H2O
  3. prostaglandin I2 synthase (prostaglandin H2)

  Catalysed by AIFM1, PYROXD1, PTGS1, PTGS2 and 1 moreendoplasmic reticulumOne of 4 routes

• prostaglandin F2alphaReach from here →
  1. A + NADH + H(+) = AH2 + NAD(+)
  2. (5Z,8Z,11Z,14Z)-eicosatetraenoate + AH2 + 2 O2 = prostaglandin H2 + A + H2O
  3. prostaglandin H2 + [thioredoxin]-dithiol = prostaglandin F2alpha + [thioredoxin]-disulfide

  Catalysed by AIFM1, PYROXD1, PTGS1, PTGS2 and 1 moreOne of 4 routes

• prostaglandin D2Reach from here →
  1. A + NADH + H(+) = AH2 + NAD(+)
  2. (5Z,8Z,11Z,14Z)-eicosatetraenoate + AH2 + 2 O2 = prostaglandin H2 + A + H2O
  3. prostaglandin-D synthase (prostaglandin H2)

  Catalysed by AIFM1, PYROXD1, PTGS1, PTGS2 and 2 morecytosolOne of 5 routes

• 15-oxo-(5Z,8Z,11Z,13E)-eicosatetraenoateReach from here →
  1. A + NADH + H(+) = AH2 + NAD(+)
  2. (15S)-hydroperoxy-(5Z,8Z,11Z,13E)-eicosatetraenoate + AH2 = (15S)-hydroxy-(5Z,8Z,11Z,13E)-eicosatetraenoate + A + H2O
  3. (15S)-15-Hydroxy-5,8,11-cis-13-trans-icosatetraenoate:NADP+ 15-oxidoreductase

  Catalysed by AIFM1, PYROXD1, TBXAS1, PTGS2 and 1 morecytosolOne of 3 routes

• D-alanineReach from here →
  1. A + NADH + H(+) = AH2 + NAD(+)
  2. L-selenocysteine + AH2 = hydrogenselenide + L-alanine + A + H(+)
  3. D-alanine(out) + L-alanine(in) = D-alanine(in) + L-alanine(out)

  Catalysed by AIFM1, PYROXD1, SCLY, SLC7A10inner side of the membrane, outer side of the membraneOne of 2 routes

• thromboxane A2Reach from here →
  1. A + NADH + H(+) = AH2 + NAD(+)
  2. (5Z,8Z,11Z,14Z)-eicosatetraenoate + AH2 + 2 O2 = prostaglandin H2 + A + H2O
  3. thromboxane A synthase (prostaglandin H2)

  Catalysed by AIFM1, PYROXD1, PTGS1, PTGS2 and 1 moreendoplasmic reticulumOne of 2 routes

• (6R)-5,10-methenyltetrahydrofolateReach from here →
  1. N(6),N(6)-dimethyl-L-lysyl(4)-[histone H3] + 2 A + 2 H2O = L-lysyl(4)-[histone H3] + 2 formaldehyde + 2 AH2
  2. formaldehyde to 5,10-methylene-THF conversion
  3. 5,10-methylenetetrahydrofolate:NAD+ oxidoreductase

  Catalysed by KDM1A, KDM1B, MTHFD2, MTHFD2Lcytosol, mitochondrial matrixOne of 4 routes

• 5-formimidoyltetrahydrofolateReach from here →
  1. uridine(34) in tRNA + 5,10-methylenetetrahydrofolate + taurine + GTP + A + H2O = 5-taurinomethyluridine(34) in tRNA + 7,8-dihydrofolate + GDP + AH2 + phosphate + H(+)
  2. 5,6,7,8-tetrahydrofolate:NAD+ oxidoreductase
  3. 5-Formiminotetrahydrofolate:L-glutamate N-formiminotransferase

  Catalysed by MTO1, DHFR, DHFR2, FTCDcytosolOne of 2 routes

• leukotriene A4Reach from here →
  1. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  2. cystine to cysteine conversion
  3. transport of GSH and leukotriene A4 (cytosol to extracellular)

  Catalysed by MOCOS, SLCO2A1cytosol, extracellular spaceOne of 2 routes

• 2-(N(omega)-L-arginino)succinateReach from here →
  1. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  2. transport of arginine and cysteine (cytosol to extracellular)
  3. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)

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

• (R)-S-lactoylglutathioneReach from here →
  1. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  2. cystine to cysteine conversion
  3. (R)-S-Lactoylglutathione methylglyoxal-lyase (isomerizing)

  Catalysed by MOCOS, GLO1, GLOD4cytosolOne of 2 routes

• 4-guanidinobutanoateReach from here →
  1. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  2. transport of arginine and cysteine (cytosol to extracellular)
  3. 4-aminobutanoate + L-arginine = 4-guanidinobutanoate + L-ornithine

  Catalysed by MOCOS, SLC7A6, GATMextracellular space, cytosol

• L-dopaReach from here →
  1. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  2. transport of cysteine and phenylalanine (cytosol to extracellular)
  3. L-dopa(out) + L-phenylalanine(in) = L-dopa(in) + L-phenylalanine(out)

  Catalysed by MOCOS, 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. (R)-malate + A = oxaloacetate + AH2
  2. L-Aspartate:2-oxoglutarate aminotransferase
  3. IMP:L-aspartate ligase (GDP-forming)

  Catalysed by D2HGDH, GOT2, ADSS2, ADSS1mitochondrial matrix, cytosolOne of 2 routes

• 3-guanidinopropanoateReach from here →
  1. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  2. transport of arginine and cysteine (cytosol to extracellular)
  3. beta-alanine + L-arginine = 3-guanidinopropanoate + L-ornithine

  Catalysed by MOCOS, SLC7A6, GATMextracellular space, cytosol

• L-histidineReach from here →
  1. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  2. transport of cysteine and phenylalanine (cytosol to extracellular)
  3. L-histidine(in) + L-phenylalanine(out) = L-histidine(out) + L-phenylalanine(in)

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

• D-ornithineReach from here →
  1. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  2. transport of cysteine and ornithine (cytosol to extracellular)
  3. D-ornithine(in) + L-ornithine(out) = D-ornithine(out) + L-ornithine(in)

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

• an N-acylglycineReach from here →
  1. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  2. transport of cysteine and glycine (cytosol to extracellular)
  3. an acyl-CoA + glycine = an N-acylglycine + CoA + H(+)

  Catalysed by MOCOS, SLC3A2, SLC7A5, SLC7A8 and 3 moreextracellular space, cytosol

• 2-methyl-3-oxopropanoateReach from here →
  1. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  2. L-cysteine hydrogen-sulfide-lyase (deaminating
  3. (R)-3-amino-2-methylpropanoate + pyruvate = 2-methyl-3-oxopropanoate + L-alanine

  Catalysed by MOCOS, CTH, AGXT2cytosol

• guanidinoacetateReach from here →
  1. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  2. transport of cysteine and glycine (cytosol to extracellular)
  3. L-Arginine:glycine amidinotransferase

  Catalysed by MOCOS, SLC3A2, SLC7A5, SLC7A8 and 1 moreextracellular space, cytosolOne of 5 routes

• sphing-4-enineReach from here →
  1. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  2. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  3. acyl-CoA:sphingosine N-acyltransferase

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

• D-threonineReach from here →
  1. A + NADH + H(+) = AH2 + NAD(+)
  2. L-selenocysteine + AH2 = hydrogenselenide + L-alanine + A + H(+)
  3. D-threonine(out) + L-alanine(in) = D-threonine(in) + L-alanine(out)

  Catalysed by AIFM1, PYROXD1, SCLY, SLC7A10inner side of the membrane, outer side of the membraneOne of 2 routes

• spermidineReach from here →
  1. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  2. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  3. diamine N-acetyltransferase (N1-acetylspermidine)

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

• hypotaurineReach from here →
  1. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  2. L-Cysteine:oxygen oxidoreductase
  3. 3-sulfino-L-alanine + H(+) = hypotaurine + CO2

  Catalysed by MOCOS, CDO1, GADL1, CSADcytosol

• L-dopaquinoneReach from here →
  1. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  2. transport of cysteine and tyrosine (cytosol to extracellular)
  3. L-Tyrosine,L-dopa:oxygen oxidoreductase

  Catalysed by MOCOS, SLC7A5, TYRextracellular space, cytosol

• glutathioneReach from here →
  1. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  2. L-cysteine hydrogen-sulfide-lyase (deaminating
  3. thiosulfate + 2 glutathione = glutathione disulfide + hydrogen sulfide + sulfite + 2 H(+)

  Catalysed by MOCOS, CTH, TSTD1cytosolOne of 4 routes

• L-threonineReach from here →
  1. A + NADH + H(+) = AH2 + NAD(+)
  2. L-selenocysteine + AH2 = hydrogenselenide + L-alanine + A + H(+)
  3. L-threonine(out) + L-alanine(in) = L-threonine(in) + L-alanine(out)

  Catalysed by AIFM1, PYROXD1, SCLY, SLC7A10inner side of the membrane, outer side of the membraneOne of 8 routes

• L-saccharopineReach from here →
  1. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  2. transport of cysteine and lysine (cytosol to extracellular)
  3. N6-(L-1,3-Dicarboxypropyl)-L-lysine:NADP+ oxidoreductase (L-lysine-forming)

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

• L-kynurenineReach from here →
  1. A + NADH + H(+) = AH2 + NAD(+)
  2. L-selenocysteine + AH2 = hydrogenselenide + L-alanine + A + H(+)
  3. L-kynurenine + H2O = anthranilate + L-alanine + H(+)

  Catalysed by AIFM1, PYROXD1, SCLY, KYNUOne of 5 routes

• beta-alanineReach from here →
  1. A + NADH + H(+) = AH2 + NAD(+)
  2. L-selenocysteine + AH2 = hydrogenselenide + L-alanine + A + H(+)
  3. beta-alanine(out) + L-alanine(in) = beta-alanine(in) + L-alanine(out)

  Catalysed by AIFM1, PYROXD1, SCLY, SLC7A10inner side of the membrane, outer side of the membraneOne of 6 routes

• leukotriene C4Reach from here →
  1. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  2. transport of cysteine and glycine (cytosol to extracellular)
  3. leukotriene C4 hydrolysis

  Catalysed by MOCOS, SLC3A2, SLC7A5, SLC7A8extracellular space, cytosolOne of 5 routes

• taurocyamineReach from here →
  1. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  2. transport of arginine and cysteine (cytosol to extracellular)
  3. taurine + L-arginine = taurocyamine + L-ornithine

  Catalysed by MOCOS, SLC7A6, GATMextracellular space, cytosol

• L-glutamate 5-semialdehydeReach from here →
  1. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  2. transport of cysteine and ornithine (cytosol to extracellular)
  3. L-ornithine + 2-oxoglutarate = L-glutamate 5-semialdehyde + L-glutamate

  Catalysed by MOCOS, SLC7A6, OATextracellular space, cytosol

• N(4)-(beta-N-acetyl-D-glucosaminyl)-L-asparagineReach from here →
  1. (R)-malate + A = oxaloacetate + AH2
  2. L-Aspartate:2-oxoglutarate aminotransferase
  3. N(4)-(beta-N-acetyl-D-glucosaminyl)-L-asparagine + H2O = N-acetyl-beta-D-glucosaminylamine + L-aspartate + H(+)

  Catalysed by D2HGDH, GOT2, AGAmitochondrial matrix

• 3-hydroxy-L-kynurenineReach from here →
  1. A + NADH + H(+) = AH2 + NAD(+)
  2. L-selenocysteine + AH2 = hydrogenselenide + L-alanine + A + H(+)
  3. 3-hydroxy-L-kynurenine + H2O = 3-hydroxyanthranilate + L-alanine + H(+)

  Catalysed by AIFM1, PYROXD1, SCLY, KYNU

• L,L-cystathionineReach from here →
  1. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  2. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  3. N-acetylglutamate synthase (N-acetyl-L-cystathionine)

  Catalysed by MOCOS, NAGSmitochondrial matrix

• 6-amino-2-oxohexanoateReach from here →
  1. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  2. transport of cysteine and lysine (cytosol to extracellular)
  3. L-lysine:oxygen 2-oxidoreductase (deaminating)

  Catalysed by MOCOS, SLC7A6extracellular space, cytosol, peroxisome

• L-homocysteineReach from here →
  1. A + NADH + H(+) = AH2 + NAD(+)
  2. N(6)-dimethylallyladenosine(37) in tRNA + (sulfur carrier)-SH + AH2 + 2 S-adenosyl-L-methionine = 2-methylsulfanyl-N(6)-dimethylallyladenosine(37) in tRNA + (sulfur carrier)-H + 5'-deoxyadenosine + L-methionine + A + S-adenosyl-L-homocysteine + 2 H(+)
  3. S-methyl-L-methionine + L-homocysteine = 2 L-methionine + H(+)

  Catalysed by AIFM1, PYROXD1, CDK5RAP1, BHMT2

• carbamoyl phosphateReach from here →
  1. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  2. transport of cysteine and glutamine (cytosol to extracellular)
  3. HCO3-:L-glutamine amido-ligase (ADP-forming, carbamate-phosphorylating)

  Catalysed by MOCOS, SLC3A2, SLC7A5, SLC7A10 and 1 moreextracellular space, cytosolOne of 4 routes

• N,N-dimethylglycineReach from here →
  1. choline + A = betaine aldehyde + AH2
  2. p-cumic alcohol:NAD+ oxidoreductase
  3. L-homocysteine + glycine betaine = N,N-dimethylglycine + L-methionine

  Catalysed by CHDH, ALDH7A1, BHMTcytosolOne of 2 routes

• S-methyl-L-methionineReach from here →
  1. A + NADH + H(+) = AH2 + NAD(+)
  2. N(6)-dimethylallyladenosine(37) in tRNA + (sulfur carrier)-SH + AH2 + 2 S-adenosyl-L-methionine = 2-methylsulfanyl-N(6)-dimethylallyladenosine(37) in tRNA + (sulfur carrier)-H + 5'-deoxyadenosine + L-methionine + A + S-adenosyl-L-homocysteine + 2 H(+)
  3. S-methyl-L-methionine + L-homocysteine = 2 L-methionine + H(+)

  Catalysed by AIFM1, PYROXD1, CDK5RAP1, BHMT2

• (2R)-2-phosphoglycerateReach from here →
  1. (R)-malate + A = oxaloacetate + AH2
  2. GTP:oxaloacetate carboxy-lyase (adding GTP
  3. 2-phospho-D-glycerate hydro-lyase (phosphoenolpyruvate-forming)

  Catalysed by D2HGDH, PCK1, ENO1, ENO2 and 1 morecytosolOne of 2 routes