L-glutamic 5-semialdehyde

Three steps

• S-sulfanylglutathioneReach from here →
  1. aldehyde dehydrogenase (1-pyrroline-5-carboxylate)
  2. L-proline + a quinone = (S)-1-pyrroline-5-carboxylate + a quinol + H(+)
  3. a quinone + hydrogen sulfide + glutathione + H(+) = S-sulfanylglutathione + a quinol

  Catalysed by ALDH4A1, PRODH, PRODH2, SQORcytosol

• N-(5Z,8Z,11Z,14Z)-eicosatetraenoyl-glycineReach from here →
  1. L-Ornithine:2-oxo-acid aminotransferase
  2. L-Arginine:glycine amidinotransferase
  3. (5Z,8Z,11Z,14Z)-eicosatetraenoyl-CoA + glycine = N-(5Z,8Z,11Z,14Z)-eicosatetraenoyl-glycine + CoA + H(+)

  Catalysed by OAT, GATM, GLYATL2mitochondrial matrix, cytosolOne of 2 routes

• N-[(R)-4-phosphopantothenoyl]-L-cysteineReach from here →
  1. L-Ornithine:2-oxo-acid aminotransferase
  2. transport of cysteine and ornithine (cytosol to extracellular)
  3. (R)-4'-phosphopantothenate:L-cysteine ligase

  Catalysed by OAT, SLC7A6, PPCSmitochondrial matrix, extracellular space, cytosolOne of 4 routes

• 3-iodo-L-tyrosineReach from here →
  1. L-Ornithine:2-oxo-acid aminotransferase
  2. transport of ornithine and tyrosine (cytosol to extracellular)
  3. thyroid peroxidase (iodine)

  Catalysed by OAT, SLC7A6, TPOmitochondrial matrix, extracellular space, cytosolOne of 2 routes

• N-benzoylglycineReach from here →
  1. L-Ornithine:2-oxo-acid aminotransferase
  2. L-Arginine:glycine amidinotransferase
  3. Hippurate (Benzoylglycinate) Formation

  Catalysed by OAT, GATMmitochondrial matrix, cytosolOne of 2 routes

• 3-sulfino-L-alanineReach from here →
  1. L-Ornithine:2-oxo-acid aminotransferase
  2. transport of cysteine and ornithine (cytosol to extracellular)
  3. L-Cysteine:oxygen oxidoreductase

  Catalysed by OAT, SLC7A6, CDO1mitochondrial matrix, extracellular space, cytosolOne of 2 routes

• L-cysteinylglycineReach from here →
  1. L-Ornithine:2-oxo-acid aminotransferase
  2. transport of alanine and ornithine (cytosol to extracellular)
  3. gamma-glutamyltransferase (alanine)

  Catalysed by OAT, SLC7A6, GGTLC3, GGT1 and 4 moremitochondrial matrix, extracellular space, cytosolOne of 2 routes

• (3R,5S)-1-pyrroline-3-hydroxy-5-carboxylateReach from here →
  1. aldehyde dehydrogenase (1-pyrroline-5-carboxylate)
  2. L-proline + a quinone = (S)-1-pyrroline-5-carboxylate + a quinol + H(+)
  3. trans-4-hydroxy-L-proline + a quinone = (3R,5S)-1-pyrroline-3-hydroxy-5-carboxylate + a quinol + H(+)

  Catalysed by ALDH4A1, PRODH, PRODH2cytosol

• N(5)-[(Z)-amino(hydroxyimino)methyl]ornithineReach from here →
  1. L-Ornithine:2-oxo-acid aminotransferase
  2. L-Arginine:glycine amidinotransferase
  3. L-arginine,NADPH:oxygen oxidoreductase (N-(omega)-hydroxyarginine-forming)

  Catalysed by OAT, GATM, NOS3, NOS1 and 1 moremitochondrial matrix, cytosol

• Mo-molybdopterinReach from here →
  1. L-Ornithine:2-oxo-acid aminotransferase
  2. transport of alanine and ornithine (cytosol to extracellular)
  3. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O

  Catalysed by OAT, SLC7A6, MOCOSmitochondrial matrix, extracellular space, cytosol

• (2S)-2-aminobutanoateReach from here →
  1. L-Ornithine:2-oxo-acid aminotransferase
  2. L-Arginine:glycine amidinotransferase
  3. (2S)-2-aminobutanoate + glyoxylate = 2-oxobutanoate + glycine

  Catalysed by OAT, GATM, AGXT2mitochondrial matrix, cytosolOne of 3 routes

• thio-Mo-molybdopterinReach from here →
  1. L-Ornithine:2-oxo-acid aminotransferase
  2. transport of cysteine and ornithine (cytosol to extracellular)
  3. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O

  Catalysed by OAT, SLC7A6, MOCOSmitochondrial matrix, extracellular space, cytosol

• glycodeoxycholateReach from here →
  1. L-Ornithine:2-oxo-acid aminotransferase
  2. L-Arginine:glycine amidinotransferase
  3. Glycodeoxycholate amidohydrolase

  Catalysed by OAT, GATMmitochondrial matrix, cytosolOne of 2 routes

• N-tetradecanoylglycineReach from here →
  1. L-Ornithine:2-oxo-acid aminotransferase
  2. L-Arginine:glycine amidinotransferase
  3. tetradecanoyl-CoA + glycine = N-tetradecanoylglycine + CoA + H(+)

  Catalysed by OAT, GATM, GLYATL2mitochondrial matrix, cytosol

• N-eicosanoylglycinateReach from here →
  1. L-Ornithine:2-oxo-acid aminotransferase
  2. L-Arginine:glycine amidinotransferase
  3. eicosanoyl-CoA + glycine = N-eicosanoylglycinate + CoA + H(+)

  Catalysed by OAT, GATM, BAATmitochondrial matrix, cytosol

• N-docosanoylglycineReach from here →
  1. L-Ornithine:2-oxo-acid aminotransferase
  2. L-Arginine:glycine amidinotransferase
  3. docosanoyl-CoA + glycine = N-docosanoylglycine + CoA + H(+)

  Catalysed by OAT, GATM, BAATmitochondrial matrix, cytosol

• N-hexacosanoylglycineReach from here →
  1. L-Ornithine:2-oxo-acid aminotransferase
  2. L-Arginine:glycine amidinotransferase
  3. hexacosanoyl-CoA + glycine = N-hexacosanoylglycine + CoA + H(+)

  Catalysed by OAT, GATM, BAATmitochondrial matrix, cytosol

• an N(2)-acyl-L-glutamineReach from here →
  1. L-Ornithine:2-oxo-acid aminotransferase
  2. transport of glutamine and ornithine (cytosol to extracellular)
  3. an acyl-CoA + L-glutamine = an N(2)-acyl-L-glutamine + CoA + H(+)

  Catalysed by OAT, SLC7A6, GLYATL1B, GLYATL1mitochondrial matrix, extracellular space, cytosol