A

Three steps

• isocitric acidReach from here →
  1. (R)-malate + A = oxaloacetate + AH2
  2. transport of malate and OAA (cytosol to mitochondria)
  3. Mitochondrial Carrier (Mc) Tcdb:2.A.29.7.2

  Catalysed by D2HGDH, SLC25A1mitochondrial matrix, cytosolOne of 8 routes

• (5Z,8Z,11Z,14Z)-eicosatetraenoateReach from here →
  1. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  2. cystine to cysteine conversion
  3. transport of arachidonate and GSH (cytosol to extracellular)

  Catalysed by MOCOScytosol, extracellular spaceOne of 2 routes

• L-lysineReach 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-lysine(in) + L-leucine(out) + Na(+)(out) = L-lysine(out) + L-leucine(in) + Na(+)(in)

  Catalysed by MOCOS, SLC7A9, SLC3A1, SLC7A7extracellular space, cytosol, inner side of the membrane, outer side of the membraneOne of 5 routes

• D-lysineReach 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-lysine(in) + L-ornithine(out) = D-lysine(out) + L-ornithine(in)

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

• putrescineReach 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. Putrescine Export via Putrescine/Arginine Antiporter

  Catalysed by MOCOS, SLC7A6, SLC3A2, SLC7A7extracellular space, cytosolOne of 4 routes

• D-arginineReach 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-arginine(in) + L-ornithine(out) = D-arginine(out) + L-ornithine(in)

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

• cis-aconitic acidReach from here →
  1. (R)-malate + A = oxaloacetate + AH2
  2. transport of malate and OAA (cytosol to mitochondria)
  3. Mitochondrial Carrier (Mc) Tcdb:2.A.29.7.2

  Catalysed by D2HGDH, SLC25A1mitochondrial matrix, cytosolOne of 8 routes

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

  Catalysed by D2HGDH, GOT2, CADmitochondrial matrix

• 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. 3-oxopropanoate + L-alanine = beta-alanine + pyruvate

  Catalysed by MOCOS, CTH, AGXT2cytosol

• thiosulfateReach from here →
  1. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  2. L-Cysteine:2-oxoglutarate aminotransferase
  3. 3-mercaptopyruvate:sulfide sulfurtransferase

  Catalysed by MOCOS, GOT2, GOT1, MPST and 1 morecytosolOne of 6 routes

• 4-methylthio-2-oxobutanoic acidReach 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:oxygen oxidoreductase (deaminating)

  Catalysed by AIFM1, PYROXD1, CDK5RAP1, IL4I1cytosol

• tetra-mu3-sulfido-tetrairon(2+)Reach from here →
  1. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  2. De novo synthesis of 2Fe2S
  3. Synthesis of 4Fe4S from 2Fe2S

  Catalysed by MOCOS, NDUFAB1, FDXR, FXN and 10 moremitochondrial matrix

• serotoninReach 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 serotonin (cytosol to extracellular)

  Catalysed by MOCOS, SLCO1A2, SLCO1B3cytosol, extracellular spaceOne of 3 routes

• (S)-3-methyl-2-oxopentanoateReach 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 + 2-oxoglutarate = (S)-3-methyl-2-oxopentanoate + L-glutamate

  Catalysed by MOCOS, SLC7A5, BCAT2, BCAT1extracellular space, cytosol

• 2-oxohexanoateReach 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-oxohexanoate + N(omega),N(omega)-dimethyl-L-arginine = L-2-aminohexanoate + 5-(3,3-dimethylguanidino)-2-oxopentanoate

  Catalysed by D2HGDH, AGXT2

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

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

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

  Catalysed by AIFM1, PYROXD1, SCLY, SLC7A10inner side of the membrane, outer side of the membraneOne of 11 routes

• all-trans-retinoateReach from here →
  1. all-trans-13,14-dihydroretinol + A = all-trans-retinol + AH2
  2. retinol: NADP+ oxidoreductase
  3. all-trans-retinal + NAD(+) + H2O = all-trans-retinoate + NADH + 2 H(+)

  Catalysed by RETSAT, DHRS3, RDH10, RDH11 and 8 morecytosolOne of 2 routes

• fatty acidReach from here →
  1. all-trans-13,14-dihydroretinol + A = all-trans-retinol + AH2
  2. Lecithin Retinol Acyltransferase
  3. 1-Acyl-sn-glycero-3-phosphocholine acylhydrolase

  Catalysed by RETSAT, LRAT, LYPLA1, LYPLA2 and 8 morecytosolOne of 3 routes

• 5-aminolevulinateReach 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 + H(+) = 5-aminolevulinate + CO2 + CoA

  Catalysed by MOCOS, SLC3A2, SLC7A5, SLC7A8 and 2 moreextracellular space, cytosol

• 3-methyl-2-oxovaleric acidReach 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-Leucine:2-oxoglutarate aminotransferase

  Catalysed by MOCOS, SLC7A5, BCAT1extracellular space, cytosolOne of 2 routes

• 3-(4-hydroxyphenyl)pyruvateReach 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:2-oxoglutarate aminotransferase

  Catalysed by MOCOS, SLC7A5, GOT2, GOT1 and 1 moreextracellular space, cytosolOne of 3 routes

• glycochenodeoxycholateReach 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. chenodeoxycholoyl-CoA + glycine = glycochenodeoxycholate + CoA + H(+)

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

• taurocholateReach 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 (GSH)

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

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

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

• glyoxylateReach 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 + 2-oxoglutarate = glyoxylate + L-glutamate

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

• 7alpha,25-dihydroxycholesterolReach from here →
  1. A + NADH + H(+) = AH2 + NAD(+)
  2. cholesterol + AH2 + O2 = 25-hydroxycholesterol + A + H2O
  3. cytochrome P450 (25-hydroxycholesterol)

  Catalysed by AIFM1, PYROXD1, CH25H, CYP7B1endoplasmic reticulumOne of 2 routes

• glycolithocholic 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. bile acid-CoA:amino acid N-acyltransferase (glycine)

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

• L-2-amino-3-oxobutanoic 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. Acetyl-CoA:glycine C-acetyltransferase

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

• (R)-lactic 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)-Lactate:NAD+ oxidoreductase

  Catalysed by MOCOS, CTH, LDHDcytosol

• (S)-lactic acidReach from here →
  1. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  2. L-cysteine hydrogen-sulfide-lyase (deaminating
  3. (S)-Lactate:NAD+ oxidoreductase

  Catalysed by MOCOS, CTH, LDHB, LDHAL6Bcytosol, mitochondrial matrixOne of 5 routes

• 2-iminopropanoateReach from here →
  1. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  2. L-cysteine hydrogen-sulfide-lyase (deaminating
  3. 2-iminopropanoate + H2O = pyruvate + NH4(+)

  Catalysed by MOCOS, CTH, RIDAcytosol

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

  Catalysed by RETSAT, DHRS3, RDH10, RDH11 and 3 morecytosolOne of 5 routes

• N-acetyl-beta-neuraminic acidReach from here →
  1. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  2. L-cysteine hydrogen-sulfide-lyase (deaminating
  3. N-Acetylneuraminate pyruvate-lyase (N-acetyl-D-mannosamine-forming)

  Catalysed by MOCOS, CTH, NPLcytosol

• 1,2-dihydroxy-5-(methylsulfanyl)pent-1-en-3-oneReach 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. 1,2-dihydroxy-5-(methylsulfanyl)pent-1-en-3-one + O2 = 3-(methylsulfanyl)propanoate + CO + formate + 2 H(+)

  Catalysed by KDM1A, KDM1B, ALDH9A1, ADI1One of 2 routes

• gamma-Glu-AlaReach 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, GGT1 and 5 moreextracellular space, cytosolOne of 2 routes

• S(8)-aminomethyldihydrolipoamideReach 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. dihydrolipoyl dehydrogenase ([protein]-N6-(lipoyl)lysine)

  Catalysed by MOCOS, SLC3A2, SLC7A5, SLC7A8 and 2 moreextracellular space, cytosol, mitochondrial matrix

• taurineReach from here →
  1. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  2. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  3. bile acid-CoA:amino acid N-acyltransferase (taurodeoxycholate)

  Catalysed by MOCOS, NAGS, BAATmitochondrial matrix, cytosolOne of 4 routes

• malonaldehydeReach 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 (12(S)-HHT)

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

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

  Catalysed by MOCOS, CTH, FAHD1cytosol

• (S)-malyl-CoAReach from here →
  1. (R)-malate + A = oxaloacetate + AH2
  2. transport of malate and OAA (cytosol to mitochondria)
  3. (S)-malate + ATP + CoA = (S)-malyl-CoA + ADP + phosphate

  Catalysed by D2HGDH, SUCLG1, SUCLA2mitochondrial matrix, cytosol

• (R)-methylmalonyl-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. aldehyde dehydrogenase (2-methyl-3-oxopropanoate)

  Catalysed by MOCOS, NAGS, ALDH6A1mitochondrial matrixOne of 2 routes

• (S)-methylmalonyl-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. (S)-methylmalonyl-CoA hydrolase

  Catalysed by MOCOS, NAGSmitochondrial matrix

• 3'-dephospho-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. acid phosphatase 2, lysosomal (CoA)

  Catalysed by MOCOS, NAGS, ACP2mitochondrial matrix, lysosomeOne of 2 routes

• (2E)-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. (2E)-dodecenoyl-CoA formation

  Catalysed by MOCOS, NAGSmitochondrial matrixOne of 3 routes

• 4,8,12-trimethyltridecanoyl-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. propanoyl-CoA C-acyltransferase (3-ketopristanoyl-CoA)

  Catalysed by MOCOS, NAGS, ACAA1, MYO5Bmitochondrial matrix, peroxisomeOne of 2 routes

• (6Z,9Z,12Z)-octadecatrienoyl-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 (gamma-linolenate)

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

• butanoyl-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. Butanoate:CoA ligase (AMP-forming)

  Catalysed by MOCOS, NAGS, ACSM4, ACSM1 and 4 moremitochondrial matrix, cytosolOne of 9 routes

• dodecanoyl-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 (lauric acid)

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

• glutaryl-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. Production of Glutaryl Carnitine

  Catalysed by MOCOS, NAGS, CPT1A, CPT1C and 1 moremitochondrial matrix, cytosolOne of 2 routes