A

Three steps

• 5-(3,3'-dimethylguanidino)-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. N(omega),N('omega)-dimethyl-L-arginine + pyruvate = 5-(3,3'-dimethylguanidino)-2-oxopentanoate + L-alanine

  Catalysed by MOCOS, CTH, AGXT2cytosol

• 5-(3-methylguanidino)-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. N(omega)-methyl-L-arginine + pyruvate = 5-(3-methylguanidino)-2-oxopentanoate + L-alanine

  Catalysed by MOCOS, CTH, AGXT2cytosol

• (5Z)-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-7-enoyl-CoA)

  Catalysed by MOCOS, NAGS, HADHA, ACAA2 and 1 moremitochondrial matrixOne of 2 routes

• N-acetylneuraminate 9-phosphateReach from here →
  1. (R)-malate + A = oxaloacetate + AH2
  2. GTP:oxaloacetate carboxy-lyase (adding GTP
  3. N-acetylneuraminate synthase

  Catalysed by D2HGDH, PCK1, NANScytosolOne of 2 routes

• (11Z)-docosenoyl-CoA(4-)Reach 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 (cis-cetoleic acid)

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

• (7Z)-tetradecenoyl-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 ((7Z)-tetradecenoic acid)

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

• (4Z,7Z,10Z,13Z)-hexadecatetraenoyl-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 (C18:4->C16:4), Mitochondrial

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

• (7Z,10Z)-hexadecadienoyl-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 Trans (C18:2->C16:2), Mitochondrial

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

• (4Z,7Z)-decadienoyl-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 (C12:3->C10:2), Mitochondrial

  Catalysed by MOCOS, NAGS, ECHS1, ACAA2 and 1 moremitochondrial matrixOne of 2 routes

• (5Z)-octenoyl-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 (C10:2->C8:1), Mitochondrial

  Catalysed by MOCOS, NAGS, ECHS1, ACAA2 and 1 moremitochondrial matrixOne of 2 routes

• CHEBI:240943Reach 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 N-acyltransferase (2-methylbutyryl-CoA)

  Catalysed by MOCOS, SLC3A2, SLC7A5, SLC7A8 and 3 moreextracellular space, cytosol, mitochondrial matrixOne of 3 routes

• a quinolReach from here →
  1. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  2. L-cysteine hydrogen-sulfide-lyase (deaminating
  3. a quinone + hydrogen sulfide + glutathione + H(+) = S-sulfanylglutathione + a quinol

  Catalysed by MOCOS, CTH, SQORcytosolOne of 2 routes

• hypochlorous 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. Peroxidase

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

• potassium atomReach 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 (cytosol to extracellular)

  Catalysed by MOCOS, SLC6A14, SLC12A2, SLC12A1extracellular space, cytosolOne of 3 routes

• phenylacetylglycineReach 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. phenylacetyl-CoA:glycine N-phenylacetyltransferase

  Catalysed by MOCOS, SLC3A2, SLC7A5, SLC7A8 and 1 moreextracellular space, cytosolOne of 3 routes

• CHEBI:27540Reach 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 (hexanoic acid)

  Catalysed by MOCOS, NAGS, ACSM4, ACSM1 and 4 moremitochondrial matrix, cytosolOne of 6 routes

• elaidic acidReach 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 (elaidate)

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

• 5-hydroxytryptophanReach 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, Tetrahydrobiopterin:Oxygen Oxidoreductase (5-Hydroxylating)

  Catalysed by MOCOS, SLC7A5, TPH1, TPH2extracellular space, cytosolOne of 2 routes

• 5(S)-HETEReach from here →
  1. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  2. cystine to cysteine conversion
  3. Glutathione: 5-HPETE oxidoreductase

  Catalysed by MOCOS, GPX5, GPX1, GPX2 and 6 morecytosolOne of 2 routes

• 3-mercaptolactic acidReach from here →
  1. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  2. L-Cysteine:2-oxoglutarate aminotransferase
  3. 3-Mercaptolactate:NAD+ oxidoreductase

  Catalysed by MOCOS, GOT2, GOT1, LDHB and 3 morecytosol

• 2-oxopentanoateReach from here →
  1. (R)-malate + A = oxaloacetate + AH2
  2. N(omega),N(omega)-dimethyl-L-arginine + oxaloacetate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + L-aspartate
  3. 2-oxopentanoate + N(omega),N(omega)-dimethyl-L-arginine = 5-(3,3-dimethylguanidino)-2-oxopentanoate + L-2-aminopentanoate

  Catalysed by D2HGDH, AGXT2

• CHEBI:28666Reach 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. LTD4 hydrolysis

  Catalysed by MOCOS, SLC3A2, SLC7A5, SLC7A8extracellular space, cytosolOne of 3 routes

• CHEBI:28701Reach from here →
  1. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  2. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  3. Propanoyl-CoA:acetyl-CoA C-acyltransferase

  Catalysed by MOCOS, NAGS, ACAA1, SCP2 and 1 moremitochondrial matrix, peroxisomeOne of 7 routes

• pyridoxine 5'-phosphateReach 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

• 3-disulfanyl-L-alanineReach from here →
  1. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  2. thioredoxin-disulfide reductase (NADPH) (cystine)
  3. L-cystine thiocysteine-lyase (deaminating

  Catalysed by MOCOS, TXNRD1, TXNRD3, TXNRD2 and 1 morecytosol

• a fatty acidReach from here →
  1. all-trans-13,14-dihydroretinol + A = all-trans-retinol + AH2
  2. Lecithin Retinol Acyltransferase
  3. an all-trans-retinyl ester + H2O = 11-cis-retinol + a fatty acid + H(+)

  Catalysed by RETSAT, LRAT, RPE65cytosolOne of 3 routes

• Ca(2+)Reach from here →
  1. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  2. K(+)(in) + L-cysteine(out) + 3 Na(+)(out) + H(+)(out) = K(+)(out) + L-cysteine(in) + 3 Na(+)(in) + H(+)(in)
  3. Ca(2+)(out) + K(+)(out) + 4 Na(+)(in) = Ca(2+)(in) + K(+)(in) + 4 Na(+)(out)

  Catalysed by MOCOS, SLC1A1, SLC24A1, SLC24A5 and 3 moreinner side of the membrane, outer side of the membraneOne of 2 routes

• Phe-tRNA(Phe)Reach 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:tRNA(Ala) ligase (AMP-forming)

  Catalysed by MOCOS, SLC7A5, FARSB, FARSAextracellular space, cytosol

• Gly-tRNA(Gly)Reach 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:tRNA(Gly) ligase (AMP-forming)

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

• Asp-tRNA(Asp)Reach from here →
  1. (R)-malate + A = oxaloacetate + AH2
  2. L-Aspartate:2-oxoglutarate aminotransferase
  3. L-Aspartate:tRNA(Asp) ligase (AMP-forming)

  Catalysed by D2HGDH, GOT2, DARS1mitochondrial matrix, cytosol

• Trp-tRNA(Trp)Reach 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 -tRNA(Trp) ligase (AMP-forming)

  Catalysed by MOCOS, SLC7A5, WARS1extracellular space, cytosol

• Ile-tRNA(Ile)Reach from here →
  1. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  2. transport of cysteine and isoleucine (cytosol to extracellular)
  3. L-Isoleucine:tRNA(Ile) ligase (AMP-forming)

  Catalysed by MOCOS, SLC7A5, IARS1extracellular space, cytosol

• Gln-tRNA(Gln)Reach 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:tRNA(Gln) ligase (AMP-forming)

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

• isocyanateReach from here →
  1. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  2. transport of citrulline and cysteine (cytosol to extracellular)
  3. L-citrulline + H(+) = isocyanate + L-ornithine

  Catalysed by MOCOS, SLC7A6, LACC1extracellular space, cytosol

• hypoioditeReach from here →
  1. A + NADH + H(+) = AH2 + NAD(+)
  2. 3,3',5'-triiodo-L-thyronine + iodide + A + H(+) = L-thyroxine + AH2
  3. iodide + H2O2 = hypoiodite + H2O

  Catalysed by AIFM1, PYROXD1, DIO1, DIO3 and 2 more

• a thiolReach from here →
  1. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  2. L-cysteine hydrogen-sulfide-lyase (deaminating
  3. L-cysteinyl-[sulfatase] + 2 a thiol + O2 = an organic disulfide + 3-oxo-L-alanyl-[sulfatase] + hydrogen sulfide + H2O + H(+)

  Catalysed by MOCOS, CTH, SUMF1cytosol

• Asn-tRNA(Asn)Reach from here →
  1. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  2. transport of asparagine and cysteine (cytosol to extracellular)
  3. L-Asparagine:tRNA(Asn) ligase (AMP-forming)

  Catalysed by MOCOS, SLC7A5, NARS1extracellular space, cytosol

• glycocholateReach 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. bile acid-CoA: amino acid N-acyltransferase

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

• cholateReach from here →
  1. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  2. cystine to cysteine conversion
  3. solute carrier organic anion transporter (cholate)

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

• D-aspartateReach from here →
  1. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  2. K(+)(in) + L-cysteine(out) + 3 Na(+)(out) + H(+)(out) = K(+)(out) + L-cysteine(in) + 3 Na(+)(in) + H(+)(in)
  3. D-aspartate(out) + K(+)(in) + 3 Na(+)(out) + H(+)(out) = D-aspartate(in) + K(+)(out) + 3 Na(+)(in) + H(+)(in)

  Catalysed by MOCOS, SLC1A1, SLC1A7, SLC1A3 and 2 moreinner side of the membrane, outer side of the membraneOne of 2 routes

• acetateReach 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 acetate and pyruvate (cytosol to extracellular)

  Catalysed by MOCOS, CTH, SLC16A4, SLC16A5 and 3 morecytosol, extracellular spaceOne of 2 routes

• CHEBI:30249Reach 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:oxygen 2,3-oxidoreductase (decyclizing)

  Catalysed by MOCOS, SLC7A5, IDO1, TDO2 and 1 moreextracellular space, cytosol

• oxalateReach 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. oxalate(in) + formate(out) = oxalate(out) + formate(in)

  Catalysed by KDM1A, KDM1B, ALDH9A1, SLC26A6inner side of the membrane, outer side of the membraneOne of 2 routes

• formic 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 formate and pyruvate (cytosol to extracellular)

  Catalysed by MOCOS, CTH, SLC16A4, SLC16A5 and 3 morecytosol, extracellular spaceOne of 5 routes

• propionic 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 propanoate and pyruvate (cytosol to extracellular)

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

• butyric 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 butyrate and pyruvate (cytosol to extracellular)

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

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

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

• malonic 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. Malonate Transport, Antiport with Chloride

  Catalysed by MOCOS, SLC6A14extracellular space, cytosolOne of 2 routes

• (9Z)-octadecenoateReach from here →
  1. all-trans-13,14-dihydroretinol + A = all-trans-retinol + AH2
  2. 1,2,3-tri-(9Z-octadecenoyl)-glycerol + all-trans-retinol = all-trans-retinyl 9Z-octadecenoate + di-(9Z)-octadecenoylglycerol
  3. di-(9Z)-octadecenoylglycerol + H2O = (9Z-octadecenoyl)-glycerol + (9Z)-octadecenoate + H(+)

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

• 3-hydroxypyruvic acidReach 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 aminotransferase

  Catalysed by MOCOS, CTH, AGXTcytosolOne of 2 routes