thio-Mo-molybdopterin

Three steps

• 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

• a 2,3-dehydroacyl-CoAReach from here →
  1. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  2. A + NADH + H(+) = AH2 + NAD(+)
  3. a 2,3-saturated acyl-CoA + A = a 2,3-dehydroacyl-CoA + AH2

  Catalysed by MOCOS, AIFM1, PYROXD1, ACAD10One of 2 routes

• L-prolineReach from here →
  1. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  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 MOCOS, SCLY, SLC1A4inner side of the membrane, outer side of the membraneOne of 2 routes

• prostaglandin E2Reach from here →
  1. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  2. (5Z,8Z,11Z,14Z)-eicosatetraenoate + AH2 + 2 O2 = prostaglandin H2 + A + H2O
  3. prostaglandin E synthase (prostaglandin H2)

  Catalysed by MOCOS, PTGS1, PTGS2, PTGES and 2 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. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  2. (9Z,12Z)-octadecadienoate + AH2 + O2 = 9-hydroxy-(10E,12Z)-octadecadienoate + A + H2O
  3. prostaglandin-endoperoxide synthase (9(S)-HPODE)

  Catalysed by MOCOS, 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. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  2. L-selenocysteine + AH2 = hydrogenselenide + L-alanine + A + H(+)
  3. gamma-glutamyltransferase (alanine)

  Catalysed by MOCOS, SCLY, GGTLC3, GGT1 and 4 morecytosolOne of 6 routes

• Fe(II)-heme aReach from here →
  1. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  2. A + NADH + H(+) = AH2 + NAD(+)
  3. Fe(II)-heme o + 2 A + H2O = Fe(II)-heme a + 2 AH2

  Catalysed by MOCOS, AIFM1, PYROXD1, COX15

• (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

• 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

• (4S)-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. (4S)-4-hydroxy-2-oxoglutarate = glyoxylate + pyruvate

  Catalysed by MOCOS, CTH, HOGA1cytosol

• (R)-noradrenalineReach 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 noradrenaline (cytosol to extracellular)

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

• (5Z,8Z,11Z,14Z,17Z)-eicosapentaenoyl-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 (EPA)

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

• (7Z,10Z,13Z,16Z,19Z)-docosapentaenoyl-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 (DPA)

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

• (9Z,12Z,15Z)-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 (linolenate)

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

• (13Z)-docosenoyl-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 (cis-erucic acid)

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

• (11Z)-eicosenoyl-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 (cis-gondoic acid)

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

• (9Z,12Z,15Z,18Z,21Z)-tetracosapentaenoyl-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 ((9Z,12Z,15Z,18Z,21Z)-TPA)

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

• (6Z,9Z,12Z,15Z,18Z,21Z)-tetracosahexaenoyl-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 ((6Z,9Z,12Z,15Z,18Z,21Z)-THA)

  Catalysed by MOCOS, NAGS, SLC27A2, ACSL4 and 6 moremitochondrial matrix, cytosolOne of 4 routes

• (15Z)-tetracosenoyl-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 (nervonic acid)

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

• (17Z)-hexacosenoyl-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 (ximenic acid)

  Catalysed by MOCOS, NAGS, SLC27A2, ACSL4 and 6 moremitochondrial matrix, cytosolOne of 4 routes

• (8Z,11Z,14Z)-eicosatrienoyl-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 (dihomo-gamma-linolenate)

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

• (8Z,11Z,14Z,17Z)-eicosatetraenoyl-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 (omega-3-arachidonic acid)

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

• (4Z,7Z,10Z,13Z,16Z,19Z)-docosahexaenoyl-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 (DHA)

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

• heptadecanoyl-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 (margaric acid)

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

• (9Z)-heptadecenoyl-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 (9-heptadecylenic acid)

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

• (11Z,14Z,17Z)-eicosatrienoyl-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 ((11Z,14Z,17Z)-eicosatrienoic acid)

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

• (2S)-2-aminobutanoateReach from here →
  1. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  2. L-selenocysteine + AH2 = hydrogenselenide + L-alanine + A + H(+)
  3. 2-oxobutanoate + L-alanine = (2S)-2-aminobutanoate + pyruvate

  Catalysed by MOCOS, SCLY, AGXT2One of 2 routes

• nonadecanoyl-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 (nonadecylic acid)

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

• (11Z)-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. Transport of 11-Octadecenoyl Carnitine into the Mitochondrial Matrix

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

• dodecanedioyl-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. Activation of dodecanedioic acid

  Catalysed by MOCOS, NAGSmitochondrial matrix, cytosolOne of 4 routes

• decanedioyl-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 Sebacoylcarnitine

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

• octanedioyl-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 Suberylcarnitine

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

• hexanedioyl-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. Activation of adipic acid for Formation of Adipoyl Carnitine

  Catalysed by MOCOS, NAGSmitochondrial matrix, cytosolOne of 4 routes

• (4Z,7Z,10Z,13Z,16Z)-docosapentaenoyl-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 ((4Z,7Z,10Z,13Z,16Z)-DPA)

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

• (11Z,14Z)-eicosadienoyl-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 ((11Z,14Z)-eicosadienoic acid)

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

• (R)-3-hydroxy-(11Z)-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. Production of 3-Hydroxyoctadecenoylcarnitinec

  Catalysed by MOCOS, NAGS, CPT1A, CPT1C and 1 moremitochondrial matrix, cytosol

• (13Z)-eicosenoyl-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 ((13Z)-eicosenoic acid)

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