A

Three steps

• phenylacetyl-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. Phenylacetate:CoA ligase

  Catalysed by MOCOS, NAGSmitochondrial matrix, cytosolOne of 3 routes

• propionyl-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. Propanoate:CoA ligase (AMP-forming)

  Catalysed by MOCOS, NAGS, ACSS3, ACSS2 and 1 moremitochondrial matrix, cytosolOne of 43 routes

• D-threo-isocitrateReach from here →
  1. (R)-malate + A = oxaloacetate + AH2
  2. acetyl-CoA:oxaloacetate C-acetyltransferase (thioester-hydrolysing)
  3. citrate = D-threo-isocitrate

  Catalysed by D2HGDH, CS, ACO1, ACO2mitochondrial matrixOne of 3 routes

• CHEBI:15570Reach from here →
  1. A + NADH + H(+) = AH2 + NAD(+)
  2. L-selenocysteine + AH2 = hydrogenselenide + L-alanine + A + H(+)
  3. alanine racemase

  Catalysed by AIFM1, PYROXD1, SCLYcytosolOne of 5 routes

• CHEBI:15611Reach 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. S-Adenosyl-L-methionine:glycine N-methyltransferase

  Catalysed by MOCOS, SLC3A2, SLC7A5, SLC7A8 and 1 moreextracellular space, cytosolOne of 4 routes

• 15(S)-HPETEReach 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. Glutathione: 15-HPETE oxidoreductase

  Catalysed by AIFM1, PYROXD1, TBXAS1, PTGS2 and 10 morecytosolOne of 2 routes

• (6R)-5,10-methylene-5,6,7,8-tetrahydrofolateReach 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. (6S)-5,6,7,8-tetrahydrofolate + NADP(+) = 7,8-dihydrofolate + NADPH + H(+)
  3. (6R)-5,10-methylene-5,6,7,8-tetrahydrofolate + glycine + H2O = (6S)-5,6,7,8-tetrahydrofolate + L-serine

  Catalysed by MTO1, DHFR, DHFR2, SHMT1 and 1 moreOne of 2 routes

• 10-formyltetrahydrofolic acidReach 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. Formate:tetrahydrofolate ligase (ADP-forming)

  Catalysed by MTO1, DHFR, DHFR2, MTHFD1cytosolOne of 3 routes

• 5-methyltetrahydrofolic acidReach 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-methyltetrahydrofolate:NADP+ oxidoreductase

  Catalysed by KDM1A, KDM1B, MTHFRcytosolOne of 3 routes

• (4aS,6R)-4a-hydroxy-L-erythro-5,6,7,8-tetrahydrobiopterinReach from here →
  1. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  2. transport of cysteine 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 MOCOS, SLC7A5, TPH1, TPH2extracellular space, cytosolOne of 2 routes

• CHEBI:15650Reach 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-acetyl-LTE4 to leukotriene E4 conversion

  Catalysed by MOCOS, NAGS, ZDHHC11B, NAA10 and 30 moremitochondrial matrix, cytosolOne of 4 routes

• trans-aconitateReach from here →
  1. (R)-malate + A = oxaloacetate + AH2
  2. acetyl-CoA:oxaloacetate C-acetyltransferase (thioester-hydrolysing)
  3. trans-aconitate(in) + citrate(out) = trans-aconitate(out) + citrate(in)

  Catalysed by D2HGDH, CS, SLC25A1mitochondrial matrix, inner side of the membrane, outer side of the membraneOne of 2 routes

• trimethylenediamineReach 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. deoxyhypusine synthase (1,3-diaminopropane)

  Catalysed by MOCOS, SLC7A6, DHPSextracellular space, cytosol

• CHEBI:15727Reach 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-alpha-acetyltransferase 10, NatA catalytic subunit (acetylcarnosine)

  Catalysed by MOCOS, NAGS, NAA10mitochondrial matrix, cytosolOne of 2 routes

• CHEBI:15746Reach 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-acetylspermine)

  Catalysed by MOCOS, NAGS, SAT1, SAT2mitochondrial matrix, cytosolOne of 2 routes

• CHEBI:15765Reach 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,tetrahydrobiopterin:oxygen oxidoreductase (3-hydroxylating)

  Catalysed by MOCOS, SLC7A5, THextracellular space, cytosolOne of 5 routes

• malonateReach from here →
  1. (R)-malate + A = oxaloacetate + AH2
  2. transport of malate and OAA (cytosol to mitochondria)
  3. malonate(out) + (S)-malate(in) = malonate(in) + (S)-malate(out)

  Catalysed by D2HGDH, SLC25A10mitochondrial matrix, cytosol, inner side of the membrane, outer side of the membraneOne of 2 routes

• bromideReach from here →
  1. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  2. cystine to cysteine conversion
  3. 2-bromoacetaldehyde:glutathione S-(formylmethyl)transferase

  Catalysed by MOCOS, MGST3, GSTA4, GSTA1 and 14 morecytosolOne of 2 routes

• N-carbamoyl-L-aspartic acidReach from here →
  1. (R)-malate + A = oxaloacetate + AH2
  2. L-Aspartate:2-oxoglutarate aminotransferase
  3. carbamoyl-phosphate:L-aspartate carbamoyltransferase

  Catalysed by D2HGDH, GOT2, CADmitochondrial matrix, cytosol

• alpha-D-glucosamine 6-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. L-glutamine:D-fructose-6-phosphate isomerase (deaminating)

  Catalysed by MOCOS, SLC3A2, SLC7A5, SLC7A10 and 2 moreextracellular space, cytosol

• thioredoxin dithiolReach from here →
  1. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  2. L-Cysteine:tRNA(Cys) ligase (AMP-forming)
  3. glycyl-tRNA(gly) phosphorylation

  Catalysed by MOCOS, CARS1cytosolOne of 2 routes

• 11-cis-retinalReach from here →
  1. all-trans-13,14-dihydroretinol + A = all-trans-retinol + AH2
  2. retinol: NADP+ oxidoreductase
  3. all-trans-Retinal 11-cis-trans-isomerase

  Catalysed by RETSAT, DHRS3, RDH10, RDH11 and 5 morecytosolOne of 5 routes

• 1,2-diacyl-sn-glycero-3-phosphocholine(1+)Reach from here →
  1. all-trans-13,14-dihydroretinol + A = all-trans-retinol + AH2
  2. Lecithin Retinol Acyltransferase
  3. Acyl-CoA:1-acyl-sn-glycero-3-phosphocholine O-acyltransferase

  Catalysed by RETSAT, LRAT, LPCAT4, LPCAT3 and 2 morecytosol

• selenophosphateReach from here →
  1. A + NADH + H(+) = AH2 + NAD(+)
  2. L-selenocysteine + AH2 = hydrogenselenide + L-alanine + A + H(+)
  3. hydrogenselenide + ATP + H2O = selenophosphate + AMP + phosphate + 2 H(+)

  Catalysed by AIFM1, PYROXD1, SCLY, SEPHS1 and 1 more

• sulfateReach from here →
  1. A + NADH + H(+) = AH2 + NAD(+)
  2. 3,3'-diiodo-L-thyronine sulfate + iodide + A + H(+) = 3,3',5-triiodo-L-thyronine sulfate + AH2
  3. Steryl-Sulfatase

  Catalysed by AIFM1, PYROXD1, DIO1, STSGolgi apparatusOne of 13 routes

• ureaReach 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. L-Arginine amidinohydrolase

  Catalysed by MOCOS, SLC7A6, ARG1extracellular space, cytosolOne of 3 routes

• 11-cis-retinyl hexadecanoateReach from here →
  1. all-trans-13,14-dihydroretinol + A = all-trans-retinol + AH2
  2. all-trans-Retinol 11-cis-trans-isomerase
  3. Palmitoyl-CoA:retinol O-acyltransferase

  Catalysed by RETSAT, DGAT1, LRAT, AWAT1 and 2 morecytosolOne of 2 routes

• 2-methyl-3-oxopropanoic acidReach 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 aminotransferase

  Catalysed by MOCOS, CTH, AGXT2cytosol, mitochondrial matrix

• CHEBI:16284Reach from here →
  1. (R)-malate + A = oxaloacetate + AH2
  2. GTP:oxaloacetate carboxy-lyase (adding GTP
  3. dATP:pyruvate 2-O-phosphotransferase

  Catalysed by D2HGDH, PCK1, PKM, PKLRcytosol

• nitriteReach from here →
  1. A + NADH + H(+) = AH2 + NAD(+)
  2. 2 nitrite + AH2 + 2 H(+) = 2 nitric oxide + A + 2 H2O
  3. 4 nitric oxide + O2 + 2 H2O = 4 nitrite + 4 H(+)

  Catalysed by AIFM1, PYROXD1, SUOX, CP

• (R)-3-aminoisobutyric acidReach from here →
  1. A + NADH + H(+) = AH2 + NAD(+)
  2. L-selenocysteine + AH2 = hydrogenselenide + L-alanine + A + H(+)
  3. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase

  Catalysed by AIFM1, PYROXD1, SCLY, AGXT2mitochondrial matrix

• lithocholic acidReach 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 lithocholate (cytosol to extracellular)

  Catalysed by MOCOS, SLCO1A2, SLCO1B3cytosol, extracellular spaceOne of 2 routes

• (R)-carnitineReach from here →
  1. choline + A = betaine aldehyde + AH2
  2. p-cumic alcohol:NAD+ oxidoreductase
  3. glycine betaine(out) + (R)-carnitine(in) = glycine betaine(in) + (R)-carnitine(out)

  Catalysed by CHDH, ALDH7A1, SLC22A5cytosol, inner side of the membrane, outer side of the membraneOne of 2 routes

• cis-aconitateReach from here →
  1. (R)-malate + A = oxaloacetate + AH2
  2. acetyl-CoA:oxaloacetate C-acetyltransferase (thioester-hydrolysing)
  3. cis-aconitate(in) + citrate(out) = cis-aconitate(out) + citrate(in)

  Catalysed by D2HGDH, CS, SLC25A1mitochondrial matrix, inner side of the membrane, outer side of the membraneOne of 2 routes

• CHEBI:16497Reach from here →
  1. (R)-malate + A = oxaloacetate + AH2
  2. GTP:oxaloacetate carboxy-lyase (adding GTP
  3. dGTP:pyruvate 2-O-phosphotransferase

  Catalysed by D2HGDH, PCK1, PKM, PKLRcytosol

• CHEBI:16523Reach 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. transport of D-serine and glycine (cytosol to extracellular)

  Catalysed by MOCOS, SLC3A2, SLC7A5, SLC7A8 and 1 moreextracellular space, cytosolOne of 4 routes

• N(2)-acetyl-L-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. N2-Acetyl-L-ornithine amidohydrolase

  Catalysed by MOCOS, SLC7A6, ACY1extracellular space, cytosol

• 2-phenylacetamideReach 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-phenylalanine:oxygen 2-oxidoreductase (decarboxylating)

  Catalysed by MOCOS, SLC7A5, EPXextracellular space, cytosol

• CHEBI:16633Reach from here →
  1. A + NADH + H(+) = AH2 + NAD(+)
  2. L-selenocysteine + AH2 = hydrogenselenide + L-alanine + A + H(+)
  3. L-selenocysteine selenide-lyase (L-alanine-forming)

  Catalysed by AIFM1, PYROXD1, SCLYcytosol

• Met-tRNA(Met)Reach 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. L-Methionine:tRNAMet ligase (AMP-forming)

  Catalysed by AIFM1, PYROXD1, CDK5RAP1, MARS1cytosol

• (S)-lactateReach from here →
  1. A + NADH + H(+) = AH2 + NAD(+)
  2. (5Z,8Z,11Z,14Z)-eicosatetraenoate + AH2 + 2 O2 = prostaglandin H2 + A + H2O
  3. prostaglandin H2(out) + 2 (S)-lactate(in) = prostaglandin H2(in) + 2 (S)-lactate(out)

  Catalysed by AIFM1, PYROXD1, PTGS1, PTGS2 and 1 moreinner side of the membrane, outer side of the membraneOne of 3 routes

• CHEBI:16668Reach 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 carboxy-lyase

  Catalysed by MOCOS, CDO1, GAD1, CSADcytosol

• 4-methylsulfanyl-2-oxobutanoateReach 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. glyoxylate + L-methionine = 4-methylsulfanyl-2-oxobutanoate + glycine

  Catalysed by AIFM1, PYROXD1, CDK5RAP1, AADAT

• N-[(S)-lactoyl]-L-phenylalanineReach 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. (S)-lactate + L-phenylalanine = N-[(S)-lactoyl]-L-phenylalanine + H2O

  Catalysed by MOCOS, SLC7A5, CNDP2extracellular space, cytosol

• 2-oxobutanoateReach from here →
  1. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  2. L-cysteine hydrogen-sulfide-lyase (deaminating
  3. 2-oxobutanoate + L-alanine = (2S)-2-aminobutanoate + pyruvate

  Catalysed by MOCOS, CTH, AGXT2cytosolOne of 3 routes

• tryptamineReach from here →
  1. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  2. transport of cysteine and tryptophan (cytosol to extracellular)
  3. L-tryptophan decarboxy-lyase

  Catalysed by MOCOS, SLC7A5, DDCextracellular space, cytosol

• 2-oxoglutaramateReach from here →
  1. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  2. L-cysteine hydrogen-sulfide-lyase (deaminating
  3. L-Glutamine:pyruvate aminotransferase

  Catalysed by MOCOS, CTH, KYAT1cytosolOne of 5 routes

• 3-fumarylpyruvateReach from here →
  1. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  2. L-cysteine hydrogen-sulfide-lyase (deaminating
  3. 3-fumarylpyruvate + H2O = fumarate + pyruvate + H(+)

  Catalysed by MOCOS, CTH, FAHD1cytosol

• pantetheine 4'-phosphateReach from here →
  1. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  2. (R)-4'-phosphopantothenate:L-cysteine ligase
  3. N-[(R)-4'-Phosphopantothenoyl]-L-cysteine carboxy-lyase

  Catalysed by MOCOS, PPCS, PPCDCcytosolOne of 2 routes

• sn-glycerol 3-phosphocholineReach from here →
  1. A + NADH + H(+) = AH2 + NAD(+)
  2. 2-(5Z,8Z,11Z,14Z)-eicosatetraenoyl-sn-glycero-3-phosphocholine + AH2 + O2 = 2-[(11R)-hydroxy-(5Z,8Z,12E,14Z)-eicosatetraenoyl]-sn-glycero-3-phosphocholine + A + H2O
  3. 2-[(11R)-hydroxy-(5Z,8Z,12E,14Z)-eicosatetraenoyl]-sn-glycero-3-phosphocholine + H2O = (11R)-hydroxy-(5Z,8Z,12E,14Z)-eicosatetraenoate + sn-glycerol 3-phosphocholine + H(+)

  Catalysed by AIFM1, PYROXD1, PTGS2, PLA2G4AOne of 6 routes