Mo-molybdopterin

Three steps

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

  Catalysed by MOCOS, AIFM1, PYROXD1, SCLY

• glycocholateReach from here →
  1. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  2. L-Alanine:glyoxylate aminotransferase
  3. bile acid-CoA: amino acid N-acyltransferase

  Catalysed by MOCOS, AGXT, AGXT2, BAATmitochondrial matrix, peroxisomeOne of 3 routes

• hydrogen sulfideReach 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. L-cysteine hydrogen-sulfide-lyase (deaminating

  Catalysed by MOCOS, CTHcytosolOne of 2 routes

• acetateReach 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 acetate and pyruvate (cytosol to extracellular)

  Catalysed by MOCOS, AGXT2, SLC16A4, SLC16A5 and 3 moremitochondrial matrix, extracellular space, cytosolOne of 2 routes

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

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

• formic 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 formate and pyruvate (cytosol to extracellular)

  Catalysed by MOCOS, AGXT2, SLC16A4, SLC16A5 and 3 moremitochondrial matrix, extracellular space, cytosolOne of 4 routes

• anthranilic acidReach 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 hydrolase

  Catalysed by MOCOS, KYNUcytosol

• propionic 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 propanoate and pyruvate (cytosol to extracellular)

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

• citric acidReach from here →
  1. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  2. (R)-malate + A = oxaloacetate + AH2
  3. acetyl-CoA:oxaloacetate C-acetyltransferase (thioester-hydrolysing)

  Catalysed by MOCOS, D2HGDH, CSmitochondrial matrixOne of 2 routes

• butyric 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 butyrate and pyruvate (cytosol to extracellular)

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

• malonic 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. Malonate Transport, Antiport with Chloride

  Catalysed by MOCOS, SLC6A14extracellular space, cytosolOne of 2 routes

• 3-hydroxypyruvic 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. L-Serine:pyruvate aminotransferase

  Catalysed by MOCOS, AGXT2, AGXTmitochondrial matrix, cytosolOne of 2 routes

• 4-hydroxy-2-oxoglutaric acidReach from here →
  1. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  2. (R)-malate + A = oxaloacetate + AH2
  3. D-aspartate:2-oxoglutarate aminotransferase

  Catalysed by MOCOS, D2HGDH, GOT2mitochondrial matrix

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

  Catalysed by MOCOS, SLC7A5, SLC7A7extracellular space, cytosol, inner side of the membrane, outer side of the membraneOne of 7 routes

• D-lysineReach 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. 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 alanine and arginine (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 alanine 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

• N-carbamoyl-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. carbamoyl phosphate + L-aspartate = N-carbamoyl-L-aspartate + phosphate + H(+)

  Catalysed by MOCOS, AGXT2, CAD

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

  Catalysed by MOCOS, AGXT2mitochondrial matrixOne of 2 routes

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

  Catalysed by MOCOS, SLC7A5, IL4I1extracellular space, cytosol

• di-mu-sulfido-diiron(2+)Reach 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. De novo synthesis of 2Fe2S

  Catalysed by MOCOS, NDUFAB1, FDXR, FXN and 4 moremitochondrial matrix

• (S)-3-methyl-2-oxopentanoateReach from here →
  1. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  2. transport of alanine 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

• 5-aminolevulinateReach from here →
  1. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  2. L-Alanine:glyoxylate aminotransferase
  3. succinyl-CoA + glycine + H(+) = 5-aminolevulinate + CO2 + CoA

  Catalysed by MOCOS, AGXT, AGXT2, ALAS1 and 1 moremitochondrial matrix

• 3-methyl-2-oxovaleric acidReach from here →
  1. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  2. transport of alanine 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 alanine 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. L-Alanine:glyoxylate aminotransferase
  3. chenodeoxycholoyl-CoA + glycine = glycochenodeoxycholate + CoA + H(+)

  Catalysed by MOCOS, AGXT, AGXT2, BAATmitochondrial matrix

• glycochenodeoxycholic acidReach from here →
  1. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  2. L-Alanine:glyoxylate aminotransferase
  3. bile acid-CoA: amino acid N-acyltransferase

  Catalysed by MOCOS, AGXT, AGXT2, BAATmitochondrial matrix, peroxisomeOne of 3 routes

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

  Catalysed by MOCOS, KYNU

• glyoxylateReach from here →
  1. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  2. L-Alanine:glyoxylate aminotransferase
  3. glycine + 2-oxoglutarate = glyoxylate + L-glutamate

  Catalysed by MOCOS, AGXT, AGXT2, AADATmitochondrial matrixOne of 11 routes

• glycolithocholic acidReach from here →
  1. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  2. L-Alanine:glyoxylate aminotransferase
  3. bile acid-CoA:amino acid N-acyltransferase (glycine)

  Catalysed by MOCOS, AGXT, AGXT2, BAATmitochondrial matrix, cytosolOne of 2 routes

• L-2-amino-3-oxobutanoic acidReach from here →
  1. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  2. L-Alanine:glyoxylate aminotransferase
  3. Acetyl-CoA:glycine C-acetyltransferase

  Catalysed by MOCOS, AGXT, AGXT2, GCATmitochondrial matrix

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

  Catalysed by MOCOS, AGXT2, LDHDmitochondrial matrix, cytosol

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

  Catalysed by MOCOS, AGXT2, LDHB, LDHAL6Bmitochondrial matrixOne of 5 routes

• 25-hydroxycholesterolReach from here →
  1. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  2. A + NADH + H(+) = AH2 + NAD(+)
  3. cholesterol + AH2 + O2 = 25-hydroxycholesterol + A + H2O

  Catalysed by MOCOS, AIFM1, PYROXD1, CH25H

• 2-iminopropanoateReach 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-iminopropanoate + H2O = pyruvate + NH4(+)

  Catalysed by MOCOS, AGXT2, RIDAmitochondrial matrix

• all-trans-4-oxoretinolReach 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. cytochrome P450 (retinol)

  Catalysed by MOCOS, RETSAT, CYP26A1cytosol

• phosphoenolpyruvic acidReach from here →
  1. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  2. (R)-malate + A = oxaloacetate + AH2
  3. GTP:oxaloacetate carboxy-lyase (adding GTP

  Catalysed by MOCOS, D2HGDH, PCK1cytosolOne of 3 routes

• 13-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. retinol binding protein (retinol)

  Catalysed by MOCOS, RETSAT, RBP1, RBP2cytosolOne of 2 routes

• N-acetyl-beta-neuraminic 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. N-Acetylneuraminate pyruvate-lyase (N-acetyl-D-mannosamine-forming)

  Catalysed by MOCOS, AGXT2, NPLmitochondrial matrix, cytosol

• S(8)-aminomethyldihydrolipoamideReach from here →
  1. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  2. L-Alanine:glyoxylate aminotransferase
  3. dihydrolipoyl dehydrogenase ([protein]-N6-(lipoyl)lysine)

  Catalysed by MOCOS, AGXT, AGXT2, GLDC and 1 moremitochondrial matrix

• taurineReach 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. bile acid-CoA:amino acid N-acyltransferase (taurodeoxycholate)

  Catalysed by MOCOS, BAATmitochondrial matrix, cytosolOne of 4 routes

• all-trans-13,14-dihydroretinolReach from here →
  1. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  2. A + NADH + H(+) = AH2 + NAD(+)
  3. all-trans-13,14-dihydroretinol + A = all-trans-retinol + AH2

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

• 3,3',5-triiodo-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',5-triiodo-L-thyronine + iodide + A + H(+) = L-thyroxine + AH2

  Catalysed by MOCOS, AIFM1, PYROXD1, DIO1 and 1 more

• 3,3',5'-triiodo-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',5'-triiodo-L-thyronine + iodide + A + H(+) = L-thyroxine + AH2

  Catalysed by MOCOS, AIFM1, PYROXD1, DIO1 and 1 more

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

  Catalysed by MOCOS, AGXT2, FAHD1mitochondrial matrix

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

  Catalysed by MOCOS, ALDH6A1mitochondrial matrixOne of 2 routes

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

  Catalysed by MOCOSmitochondrial matrix

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

  Catalysed by MOCOS, 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. amino-acid N-acetyltransferase (N-acetyl-L-alanine)
  3. (2E)-dodecenoyl-CoA formation

  Catalysed by MOCOSmitochondrial 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. amino-acid N-acetyltransferase (N-acetyl-L-alanine)
  3. propanoyl-CoA C-acyltransferase (3-ketopristanoyl-CoA)

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