A

Three steps

• glutathione disulfideReach from here →
  1. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  2. thioredoxin-disulfide reductase (NADPH) (cystine)
  3. cystine to cysteine conversion

  Catalysed by MOCOS, TXNRD1, TXNRD3, TXNRD2cytosolOne of 14 routes

• taurolithocholate 3-sulfateReach 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 sulfotaurolithocholate (cytosol to extracellular)

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

• L-tyrosineReach 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-tyrosine + glyoxylate = 3-(4-hydroxyphenyl)pyruvate + glycine

  Catalysed by MOCOS, SLC3A2, SLC7A5, SLC7A8 and 1 moreextracellular space, cytosolOne of 4 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 13 routes

• an acyl-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. acyl-CoA:sphingosine N-acyltransferase

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

• trans-4-hydroxy-L-prolineReach from here →
  1. A + NADH + H(+) = AH2 + NAD(+)
  2. L-selenocysteine + AH2 = hydrogenselenide + L-alanine + A + H(+)
  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 AIFM1, PYROXD1, SCLY, SLC1A4inner side of the membrane, outer side of the membraneOne of 2 routes

• histamineReach 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 carboxy-lyase (histamine-forming)

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

• (2S)-2-[5-amino-1-(5-phospho-beta-D-ribosyl)imidazole-4-carboxamido]succinateReach from here →
  1. (R)-malate + A = oxaloacetate + AH2
  2. L-Aspartate:2-oxoglutarate aminotransferase
  3. 1-(5-Phosphoribosyl)-5-amino-4-carboxyimidazole:L-aspartate ligase (ADP-forming)

  Catalysed by D2HGDH, GOT2, PAICSmitochondrial matrix, cytosolOne of 2 routes

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

  Catalysed by AIFM1, PYROXD1, DIO1, DIO3 and 1 morecytosolOne of 6 routes

• kynurenateReach from here →
  1. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  2. L-Cysteine:2-oxoglutarate aminotransferase
  3. 2-oxo-3-sulfanylpropanoate + L-kynurenine = kynurenate + L-cysteine + H2O

  Catalysed by MOCOS, GOT2, GOT1, AADATcytosol

• 5-amino-1-(5-phospho-beta-D-ribosyl)imidazole-4-carboxamideReach 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. phosphoribosylaminoimidazolecarboxamide formyltransferase (10-formyl-THF)

  Catalysed by MTO1, DHFR, DHFR2, ATICcytosol

• 5-guanidino-2-oxopentanoateReach 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 + O2 + H2O = 5-guanidino-2-oxopentanoate + H2O2 + NH4(+)

  Catalysed by MOCOS, SLC7A6, IL4I1extracellular space, cytosol

• (3Z)-dodecenoyl-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. acetyl-CoA C-acyltransferase (3-oxomyrist-5-enoyl-CoA)

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

• N-formyl-L-kynurenineReach 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 = N-formyl-L-kynurenine

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

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

  Catalysed by MOCOS, CTH, CLYBLcytosol

• (25R)-3alpha,7alpha,12alpha-trihydroxy-5beta-cholestan-26-oyl-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. 3alpha,7alpha,12alpha-trihydroxy-5beta-cholestanoate:CoA ligase (AMP-forming)

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

• 5-phospho-beta-D-ribosylamineReach 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. phosphoribosyl pyrophosphate amidotransferase (glutamine)

  Catalysed by MOCOS, SLC3A2, SLC7A5, SLC7A10 and 1 moreextracellular space, cytosolOne of 2 routes

• 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. D-fructose 6-phosphate + L-glutamine = D-glucosamine 6-phosphate + L-glutamate

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

• L-methionine (S)-S-oxideReach 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. [thioredoxin]-disulfide + L-methionine + H2O = L-methionine (S)-S-oxide + [thioredoxin]-dithiol

  Catalysed by AIFM1, PYROXD1, CDK5RAP1, MSRA

• L-methionine (R)-S-oxideReach 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. [thioredoxin]-disulfide + L-methionine + H2O = L-methionine (R)-S-oxide + [thioredoxin]-dithiol

  Catalysed by AIFM1, PYROXD1, CDK5RAP1, MSRB3 and 2 more

• 5-amino-2-oxopentanoateReach from here →
  1. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  2. L-cysteine hydrogen-sulfide-lyase (deaminating
  3. L-ornithine + pyruvate = 5-amino-2-oxopentanoate + L-alanine

  Catalysed by MOCOS, CTH, AGXT2cytosolOne of 3 routes

• deoxycholoyl-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. cholate-CoA ligase (3alpha,12alpha-dihydroxy-5beta-cholanate)

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

• a (2E)-enoyl-CoAReach from here →
  1. A + NADH + H(+) = AH2 + NAD(+)
  2. a 2,3-saturated acyl-CoA + A = a 2,3-dehydroacyl-CoA + AH2
  3. a 2,3-saturated acyl-CoA + O2 = a (2E)-enoyl-CoA + H2O2

  Catalysed by AIFM1, PYROXD1, ACAD10, ACOX3 and 2 more

• S-sulfanylglutathioneReach from here →
  1. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  2. L-cysteine hydrogen-sulfide-lyase (deaminating
  3. Sulfide:glutathione,quinone oxidoreductase

  Catalysed by MOCOS, CTH, SQORcytosol, mitochondrial matrixOne of 4 routes

• N-(5Z,8Z,11Z,14Z)-eicosatetraenoyl-glycineReach 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. (5Z,8Z,11Z,14Z)-eicosatetraenoyl-CoA + glycine = N-(5Z,8Z,11Z,14Z)-eicosatetraenoyl-glycine + CoA + H(+)

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

• cyclic pyranopterin phosphateReach from here →
  1. A + NADH + H(+) = AH2 + NAD(+)
  2. GTP + AH2 + S-adenosyl-L-methionine = (8S)-3',8-cyclo-7,8-dihydroguanosine 5'-triphosphate + 5'-deoxyadenosine + L-methionine + A + H(+)
  3. (8S)-3',8-cyclo-7,8-dihydroguanosine 5'-triphosphate = cyclic pyranopterin phosphate + diphosphate

  Catalysed by AIFM1, PYROXD1, MOCS1

• 4-aminobutanoateReach 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 4-aminobutyrate and chloride (cytosol to extracellular)

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

• 3-iodo-L-tyrosineReach 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. thyroid peroxidase (iodine)

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

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

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

• glycolithocholate 3-sulfateReach 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 sulfoglycolithocholate (cytosol to extracellular)

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

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

  Catalysed by AIFM1, PYROXD1, SCLY, SLC1A4inner side of the membrane, outer side of the membraneOne of 2 routes

• prostaglandin E2Reach from here →
  1. A + NADH + H(+) = AH2 + NAD(+)
  2. (5Z,8Z,11Z,14Z)-eicosatetraenoate + AH2 + 2 O2 = prostaglandin H2 + A + H2O
  3. prostaglandin E synthase (prostaglandin H2)

  Catalysed by AIFM1, PYROXD1, PTGS1, PTGS2 and 3 morecytosolOne of 5 routes

• N-benzoylglycineReach 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. Hippurate (Benzoylglycinate) Formation

  Catalysed by MOCOS, SLC3A2, SLC7A5, SLC7A8extracellular space, cytosol, mitochondrial matrixOne of 2 routes

• (9S)-hydroperoxy-(10E,12Z)-octadecadienoateReach from here →
  1. A + NADH + H(+) = AH2 + NAD(+)
  2. (9Z,12Z)-octadecadienoate + AH2 + O2 = 9-hydroxy-(10E,12Z)-octadecadienoate + A + H2O
  3. prostaglandin-endoperoxide synthase (9(S)-HPODE)

  Catalysed by AIFM1, PYROXD1, PTGS2, PTGS1cytosol

• decanoyl-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. medium-chain acyl-CoA ligase (decanoic acid)

  Catalysed by MOCOS, NAGS, ACSM4, ACSM1 and 4 moremitochondrial matrix, cytosolOne of 7 routes

• (2E)-hexadecenoyl-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. Carnitine O-Palmitoyltransferase

  Catalysed by MOCOS, NAGS, CPT1A, CPT1C and 1 moremitochondrial matrix, cytosolOne of 4 routes

• (9Z)-hexadecenoyl-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 (palmitolate)

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

• L-cysteinylglycineReach from here →
  1. A + NADH + H(+) = AH2 + NAD(+)
  2. L-selenocysteine + AH2 = hydrogenselenide + L-alanine + A + H(+)
  3. gamma-glutamyltransferase (alanine)

  Catalysed by AIFM1, PYROXD1, SCLY, GGTLC3 and 5 morecytosolOne of 6 routes

• (R)-4'-phosphopantetheineReach 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 + H(+) = (R)-4'-phosphopantetheine + CO2

  Catalysed by MOCOS, PPCS, PPCDCcytosol

• (4R)-4-hydroxy-2-oxoglutarateReach from here →
  1. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  2. L-cysteine hydrogen-sulfide-lyase (deaminating
  3. (4R)-4-hydroxy-2-oxoglutarate = glyoxylate + pyruvate

  Catalysed by MOCOS, CTH, HOGA1cytosol

• 3-hydroxyisovaleryl-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. (3-Hydroxyisovalerylcoa->3-Hydroxyisovalerylcarnitine)

  Catalysed by MOCOS, NAGS, CPT1A, CPT1Bmitochondrial matrix, cytosol

• dTMPReach 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:dUMP C-methyltransferase

  Catalysed by KDM1A, KDM1B, TYMScytosol

• CHEBI:63544Reach 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 (adrenic acid)

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

• ubiquinol-10Reach from here →
  1. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  2. L-cysteine hydrogen-sulfide-lyase (deaminating
  3. ubiquinone-10 + hydrogen sulfide + sulfite + 2 H(+) = ubiquinol-10 + thiosulfate

  Catalysed by MOCOS, CTH, SQORcytosolOne of 2 routes

• hexacosanoyl-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 (cerotic acid)

  Catalysed by MOCOS, NAGS, SLC27A2, ACSL4 and 6 moremitochondrial matrix, cytosolOne of 5 routes

• tetracosanoyl-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 (lignocerate)

  Catalysed by MOCOS, NAGS, SLC27A2, ACSL4 and 6 moremitochondrial matrix, cytosolOne of 10 routes

• docosanoyl-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 (behenic acid)

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

• N(5)-[(Z)-amino(hydroxyimino)methyl]ornithineReach 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,NADPH:oxygen oxidoreductase (N-(omega)-hydroxyarginine-forming)

  Catalysed by MOCOS, SLC7A6, NOS3, NOS1 and 1 moreextracellular space, cytosol

• (2E)-octadecenoyl-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. Carnitine O-Palmitoyltransferase

  Catalysed by MOCOS, NAGS, CPT1A, CPT1C and 1 moremitochondrial matrix, cytosolOne of 3 routes

• (6Z,9Z,12Z,15Z)-octadecatetraenoyl-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 (stearidonic acid)

  Catalysed by MOCOS, NAGS, SLC27A2, ACSL4 and 6 moremitochondrial matrix, cytosolOne of 8 routes