Mo-molybdopterin

Three steps

• N-(9Z,12Z-octadecadienoyl)-glycineReach from here →
  1. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  2. L-Alanine:glyoxylate aminotransferase
  3. (9Z,12Z)-octadecadienoyl-CoA + glycine = N-(9Z,12Z-octadecadienoyl)-glycine + CoA + H(+)

  Catalysed by MOCOS, AGXT, AGXT2, GLYATL2mitochondrial matrix

• (7Z)-octadecenoyl-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. carnitine O-palmitoyltransferase ((7Z)-octadecenoyl-CoA)

  Catalysed by MOCOS, CPT1A, CPT1C, CPT1Bmitochondrial matrix, cytosolOne of 3 routes

• acetaldehydeReach from here →
  1. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  2. transport of alanine and threonine (cytosol to extracellular)
  3. L-threonine acetaldehyde-lyase (glycine-forming)

  Catalysed by MOCOS, SLC7A5extracellular space, cytosolOne of 2 routes

• CHEBI:15345Reach 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. Acetoacetate:CoA ligase (AMP-forming)

  Catalysed by MOCOS, AACSmitochondrial matrix, cytosolOne of 2 routes

• cholineReach 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:choline O-acetyltransferase

  Catalysed by MOCOS, CHATmitochondrial matrix, nucleusOne of 2 routes

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

  Catalysed by MOCOS, AGXT2, FAHD1mitochondrial matrix

• all-trans-retinoic 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 pyruvate and retinoate (cytosol to extracellular)

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

• 4a-hydroxytetrahydrobiopterinReach 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,tetrahydrobiopterin:oxygen oxidoreductase(5-hydroxylating)

  Catalysed by MOCOS, SLC7A5, TPH1, TPH2extracellular space, cytosolOne of 3 routes

• CHEBI:15380Reach 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. 5-aminolevulinate synthase (2-amino-3-oxoadipate)

  Catalysed by MOCOS, ALAS1, CPNE7mitochondrial matrixOne of 8 routes

• CHEBI:15477Reach 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. glycine N-acyltransferase (2-methylbutyryl-CoA)

  Catalysed by MOCOS, GLYAT, GLYATL2, GLYATL1mitochondrial matrixOne of 2 routes

• 2-methylcrotonoyl-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. Production of Tiglylcarnitine

  Catalysed by MOCOS, CPT1A, CPT1Bmitochondrial matrix, cytosolOne of 2 routes

• isobutyryl-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. acetyl-CoA C-acyltransferase (3-oxo-4-methyl-pentanoyl-CoA)

  Catalysed by MOCOS, HADHA, ACAA2, HADHBmitochondrial matrixOne of 3 routes

• 3-methylbut-2-enoyl-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. glycine-N-acyltransferase (3-methylcrotonoylglycine)

  Catalysed by MOCOS, GLYATmitochondrial matrix

• isovaleryl-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. glycine N-acyltransferase (isovalerylglycine)

  Catalysed by MOCOS, GLYAT, GLYATL2, GLYATL1mitochondrial matrixOne of 3 routes

• arachidonoyl-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. Arachidonate:CoA ligase (AMP-forming)

  Catalysed by MOCOS, SLC27A2, ACSL4, ACSL3 and 5 moremitochondrial matrix, cytosolOne of 11 routes

• CHEBI:15519Reach 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. propanoyl-CoA C-acyltransferase

  Catalysed by MOCOS, ACAA1, SCP2, MYO5Bmitochondrial matrix, peroxisomeOne of 2 routes

• palmitoyl-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. Palmitate:CoA ligase (AMP-forming)

  Catalysed by MOCOS, SLC27A2, ACSL4, ACSL3 and 5 moremitochondrial matrix, cytosolOne of 11 routes

• oleoyl-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. Transport of octadecenoyl-CoA into Mitochondrial Matrix

  Catalysed by MOCOS, CPT1A, CPT1C, CPT1Bmitochondrial matrix, cytosolOne of 11 routes

• phenylacetyl-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. Phenylacetate:CoA ligase

  Catalysed by MOCOSmitochondrial matrix, cytosolOne of 3 routes

• propionyl-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. Propanoate:CoA ligase (AMP-forming)

  Catalysed by MOCOS, ACSS3, ACSS2, ACSS1mitochondrial matrix, cytosolOne of 43 routes

• (S)-malateReach from here →
  1. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  2. (R)-malate + A = oxaloacetate + AH2
  3. transport of malate and OAA (cytosol to mitochondria)

  Catalysed by MOCOS, D2HGDHmitochondrial matrix, cytosolOne of 7 routes

• CHEBI:15611Reach from here →
  1. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  2. L-Alanine:glyoxylate aminotransferase
  3. S-Adenosyl-L-methionine:glycine N-methyltransferase

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

• (4aS,6R)-4a-hydroxy-L-erythro-5,6,7,8-tetrahydrobiopterinReach 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, cytosolOne of 2 routes

• CHEBI:15650Reach 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. N-acetyl-LTE4 to leukotriene E4 conversion

  Catalysed by MOCOS, ZDHHC11B, NAA10, ESCO2 and 29 moremitochondrial matrix, cytosolOne of 2 routes

• all-trans-3-hydroxyretinolReach 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 + 2 reduced [adrenodoxin] + O2 + 2 H(+) = all-trans-3-hydroxyretinol + 2 oxidized [adrenodoxin] + H2O

  Catalysed by MOCOS, RETSAT, CYP27C1

• trimethylenediamineReach from here →
  1. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  2. transport of alanine and lysine (cytosol to extracellular)
  3. deoxyhypusine synthase (1,3-diaminopropane)

  Catalysed by MOCOS, SLC7A6, DHPSextracellular space, cytosol

• CHEBI:15727Reach 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. N-alpha-acetyltransferase 10, NatA catalytic subunit (acetylcarnosine)

  Catalysed by MOCOS, NAA10mitochondrial matrix, cytosolOne of 3 routes

• formateReach 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. formaldehyde + NAD(+) + H2O = formate + NADH + 2 H(+)

  Catalysed by MOCOS, KDM1A, KDM1B, ALDH9A1

• CHEBI:15746Reach 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. diamine N-acetyltransferase (N1-acetylspermine)

  Catalysed by MOCOS, SAT1, SAT2mitochondrial matrix, cytosolOne of 2 routes

• CHEBI:15765Reach from here →
  1. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  2. transport of alanine and tyrosine (cytosol to extracellular)
  3. L-Tyrosine,tetrahydrobiopterin:oxygen oxidoreductase (3-hydroxylating)

  Catalysed by MOCOS, SLC7A5, THextracellular space, cytosolOne of 5 routes

• N-carbamoyl-L-aspartic acidReach from here →
  1. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  2. oxaloacetate + L-alanine = L-aspartate + pyruvate
  3. carbamoyl-phosphate:L-aspartate carbamoyltransferase

  Catalysed by MOCOS, AGXT2, CADcytosol

• alpha-D-glucosamine 6-phosphateReach 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. L-glutamine:D-fructose-6-phosphate isomerase (deaminating)

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

• thioredoxin dithiolReach from here →
  1. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  2. L-Alanine:tRNA(Ala) ligase (AMP-forming)
  3. glycyl-tRNA(gly) phosphorylation

  Catalysed by MOCOS, AARS1cytosolOne of 2 routes

• methanethiolReach 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. methanethiol + O2 + H2O = hydrogen sulfide + formaldehyde + H2O2 + H(+)

  Catalysed by MOCOS, KDM1A, KDM1B, SELENBP1

• sulfateReach 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 sulfate (cytosol to extracellular)

  Catalysed by MOCOS, SLC6A14, SLC26A6extracellular space, cytosolOne of 3 routes

• ureaReach 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 amidinohydrolase

  Catalysed by MOCOS, SLC7A6, ARG1extracellular space, cytosolOne of 3 routes

• 2-methyl-3-oxopropanoic 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. (S)-3-amino-2-methylpropionate transaminase (2-methyl-3-oxopropanoate)

  Catalysed by MOCOS, AGXT2, ABATmitochondrial matrixOne of 3 routes

• 11-cis-retinolReach 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 11-cis-trans-isomerase

  Catalysed by MOCOS, RETSATcytosolOne of 3 routes

• cob(II)alaminReach from here →
  1. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  2. A + NADH + H(+) = AH2 + NAD(+)
  3. 2 cob(II)alamin + A + 2 H2O + 2 H(+) = 2 aquacob(III)alamin + AH2

  Catalysed by MOCOS, AIFM1, PYROXD1, MTRR

• iodideReach 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 + iodide + A + H(+) = L-thyroxine + AH2

  Catalysed by MOCOS, AIFM1, PYROXD1, DIO1 and 1 moreOne of 25 routes

• nitric oxideReach from here →
  1. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  2. A + NADH + H(+) = AH2 + NAD(+)
  3. 2 nitrite + AH2 + 2 H(+) = 2 nitric oxide + A + 2 H2O

  Catalysed by MOCOS, AIFM1, PYROXD1, SUOXOne of 3 routes

• selaneReach from here →
  1. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  2. L-selenocysteine selenide-lyase (L-alanine-forming)
  3. L-selenocysteine selenide-lyase (L-alanine-forming)

  Catalysed by MOCOS, SCLYcytosolOne of 3 routes

• CHEBI:16523Reach from here →
  1. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  2. L-Alanine:glyoxylate aminotransferase
  3. transport of D-serine and glycine (cytosol to extracellular)

  Catalysed by MOCOS, AGXT, AGXT2, SLC3A2 and 1 moremitochondrial matrix, extracellular space, cytosolOne of 4 routes

• N(2)-acetyl-L-ornithineReach 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. N2-Acetyl-L-ornithine amidohydrolase

  Catalysed by MOCOS, SLC7A6, ACY1extracellular space, cytosol

• 2-phenylacetamideReach 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:oxygen 2-oxidoreductase (decarboxylating)

  Catalysed by MOCOS, SLC7A5, EPXextracellular space, cytosol

• anthranilateReach from here →
  1. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  2. L-kynurenine + H2O = anthranilate + L-alanine + H(+)
  3. L-kynurenine + H2O = anthranilate + L-alanine + H(+)

  Catalysed by MOCOS, KYNU

• D-alanyl-D-alanineReach from here →
  1. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  2. alanine racemase
  3. Formation of Alanylalanine

  Catalysed by MOCOScytosol

• Met-tRNA(Met)Reach 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. L-Methionine:tRNAMet ligase (AMP-forming)

  Catalysed by MOCOS, SLC7A5, MARS1extracellular space, cytosol

• (S)-lactateReach from here →
  1. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  2. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  3. (S)-lactate + NAD(+) = pyruvate + NADH + H(+)

  Catalysed by MOCOS, AGXT2, LDHA, LDHB and 3 moremitochondrial matrix

• 3'-deoxy-3',4'-didehydro-CTPReach from here →
  1. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  2. A + NADH + H(+) = AH2 + NAD(+)
  3. CTP + AH2 + S-adenosyl-L-methionine = 3'-deoxy-3',4'-didehydro-CTP + 5'-deoxyadenosine + L-methionine + A + H2O + H(+)

  Catalysed by MOCOS, AIFM1, PYROXD1, RSAD2