Mo-molybdopterin

Three steps

• pyridoxineReach from here →
  1. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  2. L-selenocysteine selenide-lyase (L-alanine-forming)
  3. pyridoxine-5'-phosphate phosphohydrolase

  Catalysed by MOCOS, SCLY, PHOSPHO2, PDXPcytosolOne of 2 routes

• 4-methylsulfanyl-2-oxobutanoateReach from here →
  1. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  2. transport of alanine and methionine (cytosol to extracellular)
  3. glyoxylate + L-methionine = 4-methylsulfanyl-2-oxobutanoate + glycine

  Catalysed by MOCOS, SLC7A5, AADATextracellular space, cytosol

• N-[(S)-lactoyl]-L-phenylalanineReach from here →
  1. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  2. transport of alanine and phenylalanine (cytosol to extracellular)
  3. (S)-lactate + L-phenylalanine = N-[(S)-lactoyl]-L-phenylalanine + H2O

  Catalysed by MOCOS, SLC7A5, CNDP2extracellular space, cytosol

• 2-oxobutanoateReach from here →
  1. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  2. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  3. 2-oxobutanoate + L-alanine = (2S)-2-aminobutanoate + pyruvate

  Catalysed by MOCOS, AGXT2mitochondrial matrixOne of 6 routes

• tryptamineReach from here →
  1. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  2. transport of alanine and tryptophan (cytosol to extracellular)
  3. L-tryptophan decarboxy-lyase

  Catalysed by MOCOS, SLC7A5, DDCextracellular space, cytosol

• 2-oxoglutaramateReach from here →
  1. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  2. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  3. L-Glutamine:pyruvate aminotransferase

  Catalysed by MOCOS, AGXT2, KYAT1mitochondrial matrix, cytosolOne of 5 routes

• (S)-2-hydroxyglutarateReach from here →
  1. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  2. A + NADH + H(+) = AH2 + NAD(+)
  3. (S)-2-hydroxyglutarate + A = 2-oxoglutarate + AH2

  Catalysed by MOCOS, AIFM1, PYROXD1, L2HGDH

• 3-fumarylpyruvateReach from here →
  1. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  2. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  3. 3-fumarylpyruvate + H2O = fumarate + pyruvate + H(+)

  Catalysed by MOCOS, AGXT2, FAHD1mitochondrial matrix

• CHEBI:16856Reach from here →
  1. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  2. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  3. cystine to cysteine conversion

  Catalysed by MOCOScytosolOne of 3 routes

• glyoxylic acidReach from here →
  1. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  2. L-Alanine:glyoxylate aminotransferase
  3. glycine:oxygen oxidoreductase (deaminating)

  Catalysed by MOCOS, AGXT, AGXT2, DAOmitochondrial matrix, peroxisome

• trans-1,2-dithiane-4,5-diolReach from here →
  1. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  2. L-selenocysteine selenide-lyase (L-alanine-forming)
  3. selenocysteine lyase (dithiothreitol)

  Catalysed by MOCOS, SCLYcytosol

• N-acetyl-L-aspartateReach from here →
  1. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  2. oxaloacetate + L-alanine = L-aspartate + pyruvate
  3. acetyl-CoA:L-aspartate N-acetyltransferase

  Catalysed by MOCOS, AGXT2, NAT8Lmitochondrial matrixOne of 2 routes

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

  Catalysed by MOCOS, SLC6A14, SLC26A6extracellular space, cytosol

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

  Catalysed by MOCOS, AGXT2, SLC16A4, SLC16A5 and 4 moremitochondrial matrix, extracellular space, cytosolOne of 2 routes

• 3-hydroxypyruvateReach from here →
  1. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  2. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  3. L-serine + pyruvate = 3-hydroxypyruvate + L-alanine

  Catalysed by MOCOS, AGXT2, AGXTmitochondrial matrixOne of 2 routes

• L-glutamic 5-semialdehydeReach from here →
  1. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  2. transport of alanine 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. amino-acid N-acetyltransferase (N-acetyl-L-alanine)
  3. Acetyl-CoA:arylamine N-acetyltransferase

  Catalysed by MOCOS, NAT2, NAT1mitochondrial matrix, cytosol

• aceneuramateReach from here →
  1. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  2. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  3. aceneuramate = aldehydo-N-acetyl-D-mannosamine + pyruvate

  Catalysed by MOCOS, AGXT2, NPLmitochondrial matrix

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

  Catalysed by MOCOSmitochondrial matrix, cytosolOne of 2 routes

• 5'-deoxyadenosineReach from here →
  1. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  2. A + NADH + H(+) = AH2 + NAD(+)
  3. 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(+)

  Catalysed by MOCOS, AIFM1, PYROXD1, CDK5RAP1One of 5 routes

• CHEBI:17380Reach from here →
  1. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  2. transport of alanine and tryptophan (cytosol to extracellular)
  3. Transport of 3-Hydroxy-L-Kynurenine

  Catalysed by MOCOS, SLC7A5extracellular space, cytosolOne of 3 routes

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

  Catalysed by MOCOS, SLC7A5, PRODH2extracellular space, cytosolOne of 3 routes

• agmatineReach from here →
  1. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  2. transport of alanine and arginine (cytosol to extracellular)
  3. L-arginine carboxy-lyase (agmatine-forming)

  Catalysed by MOCOS, SLC7A6extracellular space, cytosol, mitochondrial matrix

• 1-O-acyl-sn-glycero-3-phosphocholine(1+)Reach from here →
  1. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  2. all-trans-13,14-dihydroretinol + A = all-trans-retinol + AH2
  3. Lecithin Retinol Acyltransferase

  Catalysed by MOCOS, RETSAT, LRATcytosol

• 5-aminolevulinic acidReach from here →
  1. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  2. L-Alanine:glyoxylate aminotransferase
  3. succinyl-CoA:glycine C-succinyltransferase (decarboxylating)

  Catalysed by MOCOS, AGXT, AGXT2, ALAS1 and 2 moremitochondrial matrix

• all-trans-retinyl hexadecanoateReach from here →
  1. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  2. all-trans-13,14-dihydroretinol + A = all-trans-retinol + AH2
  3. all-trans-retinol + hexadecanoyl-CoA = all-trans-retinyl hexadecanoate + CoA

  Catalysed by MOCOS, RETSAT, DGAT1, PNPLA4 and 3 moreOne of 2 routes

• indole-3-pyruvateReach from here →
  1. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  2. transport of alanine 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. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  2. A + NADH + H(+) = AH2 + NAD(+)
  3. 3,3',5'-triiodo-L-thyronine sulfate + iodide + A + H(+) = L-thyroxine sulfate + AH2

  Catalysed by MOCOS, AIFM1, PYROXD1, DIO1

• 3,3'-diiodo-L-thyronineReach from here →
  1. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  2. A + NADH + H(+) = AH2 + NAD(+)
  3. 3,3'-diiodo-L-thyronine + iodide + A + H(+) = 3,3',5-triiodo-L-thyronine + AH2

  Catalysed by MOCOS, AIFM1, PYROXD1, DIO1 and 1 moreOne of 2 routes

• 3,3'-diiodo-L-thyronine sulfateReach from here →
  1. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  2. A + NADH + H(+) = AH2 + NAD(+)
  3. 3,3'-diiodo-L-thyronine sulfate + iodide + A + H(+) = 3,3',5-triiodo-L-thyronine sulfate + AH2

  Catalysed by MOCOS, AIFM1, PYROXD1, DIO1One of 2 routes

• (7Z,10Z,13Z)-hexadecatrienoyl-CoAReach from here →
  1. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  2. amino-acid N-acetyltransferase (N-acetyl-L-alanine)
  3. Fatty Acid Beta Oxidation (C18:3->C16:3), Mitochondrial

  Catalysed by MOCOS, HADHA, ACADVL, HADHBmitochondrial matrix

• CHEBI:17724Reach from here →
  1. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  2. N(6),N(6)-dimethyl-L-lysyl(4)-[histone H3] + 2 A + 2 H2O = L-lysyl(4)-[histone H3] + 2 formaldehyde + 2 AH2
  3. dimethylglycine dehydrogenase (formaldehyde)

  Catalysed by MOCOS, KDM1A, KDM1B, PDPR and 1 morecytosol

• glycine betaineReach from here →
  1. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  2. choline + A = betaine aldehyde + AH2
  3. p-cumic alcohol:NAD+ oxidoreductase

  Catalysed by MOCOS, CHDH, ALDH7A1cytosolOne of 7 routes

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

  Catalysed by MOCOS, SLC7A5, BCAT2extracellular space, cytosol, mitochondrial matrixOne of 4 routes

• all-trans-retinalReach from here →
  1. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  2. all-trans-13,14-dihydroretinol + A = all-trans-retinol + AH2
  3. retinol: NADP+ oxidoreductase

  Catalysed by MOCOS, RETSAT, DHRS3, RDH10 and 4 morecytosolOne of 6 routes

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

  Catalysed by MOCOSmitochondrial matrix, cytosolOne of 2 routes

• 3-oxopropanoic acidReach from here →
  1. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  2. beta-alanine(out) + L-alanine(in) = beta-alanine(in) + L-alanine(out)
  3. beta-alanine:2-oxoglutarate aminotransferase

  Catalysed by MOCOS, SLC7A10, ABATinner side of the membrane, outer side of the membrane, mitochondrial matrix

• 3-phenylpyruvateReach from here →
  1. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  2. transport of alanine and phenylalanine (cytosol to extracellular)
  3. Phenylalanine Transaminase, Mitochondrial

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

• a diacylglycerolReach from here →
  1. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  2. all-trans-13,14-dihydroretinol + A = all-trans-retinol + AH2
  3. a triacylglycerol + all-trans-retinol = an all-trans-retinyl ester + a diacylglycerol

  Catalysed by MOCOS, RETSAT, PNPLA4, PNPLA2

• dTDPReach from here →
  1. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  2. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  3. pyruvate kinase (dTDP)

  Catalysed by MOCOS, AGXT2, PKM, PKLRmitochondrial matrix, cytosol

• CHEBI:18183Reach from here →
  1. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  2. gamma-glutamyltransferase (alanine)
  3. gamma-glutamylcyclotransferase ((5-L-glutamyl)-L-amino acid)

  Catalysed by MOCOS, GGT1, GGT5, GGTLC2 and 5 moreextracellular space, cytosol

• N-carbamoyl-beta-alanineReach from here →
  1. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  2. transport of alanine and chloride (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. L-Alanine:glyoxylate aminotransferase
  3. 5-Phospho-D-ribosylamine:glycine ligase (ADP-forming)

  Catalysed by MOCOS, AGXT, AGXT2, GARTmitochondrial matrix, cytosol

• 2-phenylethylamineReach from here →
  1. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  2. transport of alanine and phenylalanine (cytosol to extracellular)
  3. L-phenylalanine carboxy-lyase (phenylethylamine-forming)

  Catalysed by MOCOS, SLC7A5, DDCextracellular space, cytosol

• CHEBI:18405Reach from here →
  1. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  2. L-selenocysteine selenide-lyase (L-alanine-forming)
  3. pyridoxine 5'-phosphate:oxygen oxidoreductase

  Catalysed by MOCOS, SCLY, PNPOcytosolOne of 3 routes

• 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 alanine 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

• gamma-L-glutamyl-(2S)-2-aminobutanoateReach from here →
  1. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  2. 2-oxobutanoate + L-alanine = (2S)-2-aminobutanoate + pyruvate
  3. (2S)-2-aminobutanoate + L-glutamate + ATP = gamma-L-glutamyl-(2S)-2-aminobutanoate + ADP + phosphate + H(+)

  Catalysed by MOCOS, AGXT2, GCLC

• ophthalmateReach from here →
  1. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  2. L-Alanine:glyoxylate aminotransferase
  3. gamma-L-glutamyl-(2S)-2-aminobutanoate + glycine + ATP = ophthalmate + ADP + phosphate + H(+)

  Catalysed by MOCOS, AGXT, AGXT2, GSSmitochondrial matrix