A

Three steps

• glyoxylic acidReach 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. glycine:oxygen oxidoreductase (deaminating)

  Catalysed by MOCOS, SLC3A2, SLC7A5, SLC7A8 and 1 moreextracellular space, cytosol, peroxisomeOne of 2 routes

• N-acetyl-L-aspartateReach from here →
  1. (R)-malate + A = oxaloacetate + AH2
  2. L-Aspartate:2-oxoglutarate aminotransferase
  3. acetyl-CoA:L-aspartate N-acetyltransferase

  Catalysed by D2HGDH, GOT2, NAT8Lmitochondrial matrixOne of 2 routes

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

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

• oxalic acidReach from here →
  1. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  2. transport of chloride and cysteine (cytosol to extracellular)
  3. transport of chloride and oxalate (cytosol to extracellular)

  Catalysed by MOCOS, SLC6A14, SLC26A6extracellular space, cytosolOne of 4 routes

• (R)-3-hydroxybutyric acidReach from here →
  1. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  2. L-cysteine hydrogen-sulfide-lyase (deaminating
  3. transport of (R)-3-hydroxybutanoate and pyruvate (cytosol to extracellular)

  Catalysed by MOCOS, CTH, SLC16A4, SLC16A5 and 4 morecytosol, extracellular spaceOne of 2 routes

• taurolithocholateReach 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 taurolithocholate (cytosol to extracellular)

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

• 3-hydroxypyruvateReach from here →
  1. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  2. L-cysteine hydrogen-sulfide-lyase (deaminating
  3. L-serine + pyruvate = 3-hydroxypyruvate + L-alanine

  Catalysed by MOCOS, CTH, AGXTcytosol

• CHEBI:17208Reach from here →
  1. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  2. cystine to cysteine conversion
  3. glutathione:dehydroascorbate oxidoreductase

  Catalysed by MOCOScytosol

• homocysteineReach from here →
  1. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  2. cystine to cysteine conversion
  3. Formation of Homocystine

  Catalysed by MOCOScytosol

• L-glutamic 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-oxo-acid aminotransferase

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

• anilineReach from here →
  1. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  2. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  3. Acetyl-CoA:arylamine N-acetyltransferase

  Catalysed by MOCOS, NAGS, NAT2, NAT1mitochondrial matrix, cytosol

• aceneuramateReach from here →
  1. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  2. L-cysteine hydrogen-sulfide-lyase (deaminating
  3. aceneuramate = aldehydo-N-acetyl-D-mannosamine + pyruvate

  Catalysed by MOCOS, CTH, NPLcytosol

• CHEBI:17312Reach from here →
  1. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  2. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  3. N1,N12-diacetylspermine to N1-acetylspermine conversion

  Catalysed by MOCOS, NAGSmitochondrial matrix, cytosolOne of 2 routes

• sulfiteReach from here →
  1. (R)-malate + A = oxaloacetate + AH2
  2. transport of malate and OAA (cytosol to mitochondria)
  3. transport of malate and sulfite (cytosol to mitochondria)

  Catalysed by D2HGDH, SLC25A10mitochondrial matrix, cytosolOne of 2 routes

• CHEBI:17380Reach 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. Transport of 3-Hydroxy-L-Kynurenine

  Catalysed by MOCOS, SLC7A5extracellular space, cytosolOne of 2 routes

• (S)-1-pyrroline-5-carboxylateReach from here →
  1. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  2. transport of cysteine and proline (cytosol to extracellular)
  3. L-proline:(acceptor) oxidoreductase

  Catalysed by MOCOS, SLC7A5, PRODH2extracellular space, cytosolOne of 2 routes

• a monoacylglycerolReach from here →
  1. all-trans-13,14-dihydroretinol + A = all-trans-retinol + AH2
  2. a triacylglycerol + all-trans-retinol = an all-trans-retinyl ester + a diacylglycerol
  3. a diacylglycerol + H2O = a monoacylglycerol + a fatty acid + H(+)

  Catalysed by RETSAT, PNPLA4, PNPLA2, DDHD2 and 1 more

• agmatineReach 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 carboxy-lyase (agmatine-forming)

  Catalysed by MOCOS, SLC7A6extracellular space, cytosol, mitochondrial matrix

• enol-oxaloacetateReach from here →
  1. (R)-malate + A = oxaloacetate + AH2
  2. transport of malate and OAA (cytosol to mitochondria)
  3. (S)-malate + a quinone = enol-oxaloacetate + a quinol

  Catalysed by D2HGDH, SDHB, SDHAmitochondrial matrix, cytosol

• 5-aminolevulinic acidReach 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. succinyl-CoA:glycine C-succinyltransferase (decarboxylating)

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

• diiodineReach from here →
  1. A + NADH + H(+) = AH2 + NAD(+)
  2. 3,3',5'-triiodo-L-thyronine + iodide + A + H(+) = L-thyroxine + AH2
  3. iodide:hydrogen-peroxide oxidoreductase

  Catalysed by AIFM1, PYROXD1, DIO1, DIO3 and 1 morecytosolOne of 2 routes

• nitrateReach from here →
  1. A + NADH + H(+) = AH2 + NAD(+)
  2. 2 nitrite + AH2 + 2 H(+) = 2 nitric oxide + A + 2 H2O
  3. Fe(II)-heme b-[protein] + nitric oxide + O2 = Fe(III)-heme b-[protein] + nitrate

  Catalysed by AIFM1, PYROXD1, SUOX, CYGB

• indole-3-pyruvateReach 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 + O2 + H2O = indole-3-pyruvate + H2O2 + NH4(+)

  Catalysed by MOCOS, SLC7A5, IL4I1extracellular space, cytosolOne of 2 routes

• 3,3',5-triiodo-L-thyronine sulfateReach from here →
  1. A + NADH + H(+) = AH2 + NAD(+)
  2. 3,3',5-triiodo-L-thyronine + iodide + A + H(+) = L-thyroxine + AH2
  3. aryl sulfotransferase (PAPS)

  Catalysed by AIFM1, PYROXD1, DIO1, DIO2 and 4 morecytosolOne of 2 routes

• (7Z,10Z,13Z)-hexadecatrienoyl-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. Fatty Acid Beta Oxidation (C18:3->C16:3), Mitochondrial

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

• Ala-tRNA(Ala)Reach from here →
  1. A + NADH + H(+) = AH2 + NAD(+)
  2. L-selenocysteine + AH2 = hydrogenselenide + L-alanine + A + H(+)
  3. L-Alanine:tRNA(Ala) ligase (AMP-forming)

  Catalysed by AIFM1, PYROXD1, SCLY, AARS1cytosol

• trans-urocanateReach from here →
  1. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  2. transport of cysteine and histidine (cytosol to extracellular)
  3. L-histidine ammonia-lyase (urocanate-forming)

  Catalysed by MOCOS, SLC7A6, HALextracellular space, cytosol

• 5-hydroxy-L-tryptophanReach 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, cytosol

• 4-methyl-2-oxopentanoateReach from here →
  1. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  2. transport of cysteine and leucine (cytosol to extracellular)
  3. L-Leucine:2-oxoglutarate aminotransferase

  Catalysed by MOCOS, SLC7A9, SLC3A1, BCAT2extracellular space, cytosol, mitochondrial matrixOne of 4 routes

• 7alpha-hydroxycholest-4-en-3-oneReach from here →
  1. A + NADH + H(+) = AH2 + NAD(+)
  2. cholesterol + AH2 + O2 = 25-hydroxycholesterol + A + H2O
  3. Cholest-5-Ene-3Beta, 7Alpha-Diol:NAD+ 3-Oxidoreductase Bile Acid Biosynthesis

  Catalysed by AIFM1, PYROXD1, CH25H, HSD3B7endoplasmic reticulum

• CHEBI:17927Reach from here →
  1. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  2. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  3. N1,N8-diacetylspermidine to N1-acetylspermidine conversion

  Catalysed by MOCOS, NAGSmitochondrial matrix, cytosolOne of 2 routes

• 3-phenylpyruvateReach 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. Phenylalanine Transaminase, Mitochondrial

  Catalysed by MOCOS, SLC7A5, GOT2, TATextracellular space, cytosol, mitochondrial matrixOne of 5 routes

• fumaric acidReach from here →
  1. (R)-malate + A = oxaloacetate + AH2
  2. transport of malate and OAA (cytosol to mitochondria)
  3. (S)-malate hydro-lyase (fumarate-forming)

  Catalysed by D2HGDH, FHmitochondrial matrix, cytosolOne of 5 routes

• thiocyanateReach from here →
  1. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  2. L-Cysteine:2-oxoglutarate aminotransferase
  3. 3-mercaptopyruvate sulfurtransferase (hydrogen-cyanide)

  Catalysed by MOCOS, GOT2, GOT1, MPST and 1 morecytosol

• dTDPReach from here →
  1. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  2. L-cysteine hydrogen-sulfide-lyase (deaminating
  3. pyruvate kinase (dTDP)

  Catalysed by MOCOS, CTH, PKM, PKLRcytosol

• dTTPReach from here →
  1. (R)-malate + A = oxaloacetate + AH2
  2. GTP:oxaloacetate carboxy-lyase (adding GTP
  3. pyruvate kinase (dTDP)

  Catalysed by D2HGDH, PCK1, PKM, PKLRcytosol

• CHEBI:18095Reach from here →
  1. A + NADH + H(+) = AH2 + NAD(+)
  2. L-selenocysteine + AH2 = hydrogenselenide + L-alanine + A + H(+)
  3. Transport of Hydroxy-Proline

  Catalysed by AIFM1, PYROXD1, SCLY, SLC1A4extracellular space, cytosolOne of 5 routes

• CHEBI:18183Reach from here →
  1. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  2. L-glutamate:L-cysteine gamma-ligase (ADP-forming)
  3. gamma-glutamylcyclotransferase (gamma-glutamyl-cysteine)

  Catalysed by MOCOS, GCLC, GCLM, GGCT and 1 morecytosol

• N-carbamoyl-beta-alanineReach from here →
  1. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  2. transport of chloride and cysteine (cytosol to extracellular)
  3. Transport of 3-Ureidopropionate

  Catalysed by MOCOS, SLC6A14extracellular space, cytosolOne of 2 routes

• N(1)-(5-phospho-D-ribosyl)glycinamideReach 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. 5-Phospho-D-ribosylamine:glycine ligase (ADP-forming)

  Catalysed by MOCOS, SLC3A2, SLC7A5, SLC7A8 and 1 moreextracellular space, cytosol

• 2-phenylethylamineReach 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 carboxy-lyase (phenylethylamine-forming)

  Catalysed by MOCOS, SLC7A5, DDCextracellular space, cytosol

• 2-formamido-N(1)-(5-phospho-D-ribosyl)acetamidineReach 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. 5'-Phosphoribosylformylglycinamide:L-glutamine amido-ligase (ADP-forming)

  Catalysed by MOCOS, SLC3A2, SLC7A5, SLC7A10 and 1 moreextracellular space, cytosol

• ophthalmateReach 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. gamma-L-glutamyl-(2S)-2-aminobutanoate + glycine + ATP = ophthalmate + ADP + phosphate + H(+)

  Catalysed by MOCOS, SLC3A2, SLC7A5, SLC7A8 and 1 moreextracellular space, cytosol

• S-methylmercury-L-cysteineReach 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-methylmercury-L-cysteine(out) + L-methionine(in) = S-methylmercury-L-cysteine(in) + L-methionine(out)

  Catalysed by AIFM1, PYROXD1, CDK5RAP1, SLC7A8inner side of the membrane, outer side of the membraneOne of 6 routes

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

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

• 11-cis-retinyl esterReach from here →
  1. all-trans-13,14-dihydroretinol + A = all-trans-retinol + AH2
  2. all-trans-Retinol 11-cis-trans-isomerase
  3. 11-cis-retinol + a fatty acyl-CoA = 11-cis-retinyl ester + CoA

  Catalysed by RETSAT, AWAT2cytosol

• 13-cis-retinyl esterReach from here →
  1. all-trans-13,14-dihydroretinol + A = all-trans-retinol + AH2
  2. retinol binding protein (retinol)
  3. 13-cis-retinol + a fatty acyl-CoA = 13-cis-retinyl ester + CoA

  Catalysed by RETSAT, RBP1, RBP2, AWAT2cytosol

• 9-cis-retinyl esterReach from here →
  1. all-trans-13,14-dihydroretinol + A = all-trans-retinol + AH2
  2. retinol binding protein (retinol)
  3. 9-cis-retinol + a fatty acyl-CoA = 9-cis-retinyl ester + CoA

  Catalysed by RETSAT, RBP1, RBP2, AWAT2cytosol

• (6R)-10-formyltetrahydrofolateReach 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 + NAD(+) + H2O = formate + NADH + 2 H(+)
  3. (6S)-5,6,7,8-tetrahydrofolate + formate + ATP = (6R)-10-formyltetrahydrofolate + ADP + phosphate

  Catalysed by KDM1A, KDM1B, ALDH9A1, MTHFD1 and 1 moreOne of 2 routes

• N(omega),N('omega)-dimethyl-L-arginineReach from here →
  1. A + NADH + H(+) = AH2 + NAD(+)
  2. L-selenocysteine + AH2 = hydrogenselenide + L-alanine + A + H(+)
  3. N(omega),N('omega)-dimethyl-L-arginine + pyruvate = 5-(3,3'-dimethylguanidino)-2-oxopentanoate + L-alanine

  Catalysed by AIFM1, PYROXD1, SCLY, AGXT2One of 2 routes