L-ornithine

Three steps

• phosphoenolpyruvic acidReach from here →
  1. transport of alanine and ornithine (cytosol to extracellular)
  2. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  3. pyruvate kinase (dTDP)

  Catalysed by SLC7A6, AGXT2, PKM, PKLRextracellular space, cytosol, mitochondrial matrix

• spermineReach from here →
  1. L-ornithine carboxy-lyase (putrescine-forming)
  2. S-adenosylmethioninamine:putrescine 3-aminopropyltransferase
  3. S-adenosyl 3-(methylsulfanyl)propylamine + spermidine = spermine + S-methyl-5'-thioadenosine + H(+)

  Catalysed by ODC1, SRM, SMScytosolOne of 2 routes

• N-acetyl-beta-neuraminic acidReach from here →
  1. transport of alanine and ornithine (cytosol to extracellular)
  2. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  3. N-Acetylneuraminate pyruvate-lyase (N-acetyl-D-mannosamine-forming)

  Catalysed by SLC7A6, AGXT2, NPLextracellular space, cytosol, mitochondrial matrix

• tert-butanolReach from here →
  1. L-Arginine:glycine amidinotransferase
  2. gamma-L-glutamyl-L-cysteine + glycine + ATP = glutathione + ADP + phosphate + H(+)
  3. tert-butyl hydroperoxide + 2 glutathione = tert-butanol + glutathione disulfide + H2O

  Catalysed by GATM, GSS, CLIC2, GPX1 and 3 morecytosol

• N-(acetyl)-sphing-4-enineReach from here →
  1. N2-Acetyl-L-ornithine amidohydrolase
  2. N2-Acetyl-L-ornithine amidohydrolase
  3. N-(acetyl)-sphing-4-enine + H2O = sphing-4-enine + acetate

  Catalysed by ACY1, ASAH1cytosol

• 2-methyl-1-hydroxypropylthiamine diphosphateReach from here →
  1. transport of ornithine and valine (cytosol to extracellular)
  2. L-Valine:2-oxoglutarate aminotransferase
  3. branched chain keto acid dehydrogenase E1 (3-methyl-2-oxobutyrate)

  Catalysed by SLC7A6, BCAT2, BCKDHA, BCKDHBextracellular space, cytosol, mitochondrial matrix

• 1,2-dihydroxy-5-(methylsulfanyl)pent-1-en-3-oneReach from here →
  1. transport of methionine and ornithine (cytosol to extracellular)
  2. glyoxylate + L-methionine = 4-methylsulfanyl-2-oxobutanoate + glycine
  3. 1,2-dihydroxy-5-(methylsulfanyl)pent-1-en-3-one + O2 = 4-methylsulfanyl-2-oxobutanoate + formate + 2 H(+)

  Catalysed by SLC7A6, AADAT, ADI1extracellular space, cytosol

• taurineReach from here →
  1. L-ornithine carboxy-lyase (putrescine-forming)
  2. acetyl-CoA:putrescine N-acetyltransferase
  3. bile acid-CoA:amino acid N-acyltransferase (taurodeoxycholate)

  Catalysed by ODC1, SAT1, SAT2, BAATcytosolOne of 5 routes

• all-trans-13,14-dihydroretinolReach from here →
  1. transport of alanine and ornithine (cytosol to extracellular)
  2. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  3. all-trans-13,14-dihydroretinol + A = all-trans-retinol + AH2

  Catalysed by SLC7A6, MOCOS, RETSATextracellular space, cytosol

• 3,3',5-triiodo-L-thyronineReach from here →
  1. L-Arginine:glycine amidinotransferase
  2. gamma-L-glutamyl-L-cysteine:glycine ligase (ADP-forming)
  3. transport of GSH and triiodothyronine (cytosol to extracellular)

  Catalysed by GATM, GSS, SLCO1A2, SLCO1B3cytosol, extracellular spaceOne of 8 routes

• 3,3',5'-triiodo-L-thyronineReach from here →
  1. transport of alanine and ornithine (cytosol to extracellular)
  2. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  3. 3,3',5'-triiodo-L-thyronine + iodide + A + H(+) = L-thyroxine + AH2

  Catalysed by SLC7A6, MOCOS, DIO1, DIO3extracellular space, cytosol

• a 3-acylpyruvateReach from here →
  1. transport of alanine and ornithine (cytosol to extracellular)
  2. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  3. a 3-acylpyruvate + H2O = a carboxylate + pyruvate + H(+)

  Catalysed by SLC7A6, AGXT2, FAHD1extracellular space, cytosol, mitochondrial matrix

• (R)-methylmalonyl-CoAReach from here →
  1. L-ornithine carboxy-lyase (putrescine-forming)
  2. acetyl-CoA:putrescine N-acetyltransferase
  3. aldehyde dehydrogenase (2-methyl-3-oxopropanoate)

  Catalysed by ODC1, SAT1, SAT2, ALDH6A1cytosol, mitochondrial matrixOne of 2 routes

• (S)-methylmalonyl-CoAReach from here →
  1. L-ornithine carboxy-lyase (putrescine-forming)
  2. acetyl-CoA:putrescine N-acetyltransferase
  3. (S)-methylmalonyl-CoA hydrolase

  Catalysed by ODC1, SAT1, SAT2cytosol, mitochondrial matrix

• 3'-dephospho-CoAReach from here →
  1. L-ornithine carboxy-lyase (putrescine-forming)
  2. acetyl-CoA:putrescine N-acetyltransferase
  3. acid phosphatase 2, lysosomal (CoA)

  Catalysed by ODC1, SAT1, SAT2, ACP2cytosol, lysosomeOne of 2 routes

• (2E)-dodecenoyl-CoAReach from here →
  1. L-ornithine carboxy-lyase (putrescine-forming)
  2. acetyl-CoA:putrescine N-acetyltransferase
  3. (2E)-dodecenoyl-CoA formation

  Catalysed by ODC1, SAT1, SAT2cytosol, mitochondrial matrixOne of 3 routes

• 4,8,12-trimethyltridecanoyl-CoAReach from here →
  1. L-ornithine carboxy-lyase (putrescine-forming)
  2. acetyl-CoA:putrescine N-acetyltransferase
  3. propanoyl-CoA C-acyltransferase (3-ketopristanoyl-CoA)

  Catalysed by ODC1, SAT1, SAT2, ACAA1 and 1 morecytosol, peroxisomeOne of 2 routes

• (6Z,9Z,12Z)-octadecatrienoyl-CoAReach from here →
  1. L-ornithine carboxy-lyase (putrescine-forming)
  2. acetyl-CoA:putrescine N-acetyltransferase
  3. long-chain-fatty-acid-CoA ligase (gamma-linolenate)

  Catalysed by ODC1, SAT1, SAT2, SLC27A2 and 7 morecytosolOne of 7 routes

• benzoyl-CoAReach from here →
  1. L-Arginine:glycine amidinotransferase
  2. Hippurate (Benzoylglycinate) Formation
  3. benzoyl-CoA + glycine = N-benzoylglycine + CoA + H(+)

  Catalysed by GATM, GLYATcytosol, mitochondrial matrix

• butanoyl-CoAReach from here →
  1. L-ornithine carboxy-lyase (putrescine-forming)
  2. acetyl-CoA:putrescine N-acetyltransferase
  3. Butanoate:CoA ligase (AMP-forming)

  Catalysed by ODC1, SAT1, SAT2, ACSM4 and 5 morecytosolOne of 9 routes

• dodecanoyl-CoAReach from here →
  1. L-ornithine carboxy-lyase (putrescine-forming)
  2. acetyl-CoA:putrescine N-acetyltransferase
  3. long-chain-fatty-acid-CoA ligase (lauric acid)

  Catalysed by ODC1, SAT1, SAT2, SLC27A2 and 7 morecytosolOne of 10 routes

• glutaryl-CoAReach from here →
  1. L-ornithine carboxy-lyase (putrescine-forming)
  2. acetyl-CoA:putrescine N-acetyltransferase
  3. Production of Glutaryl Carnitine

  Catalysed by ODC1, SAT1, SAT2, CPT1A and 2 morecytosolOne of 2 routes

• hexadecanoyl-CoAReach from here →
  1. L-Arginine:glycine amidinotransferase
  2. hexadecanoyl-CoA + glycine = N-hexadecanoylglycine + CoA + H(+)
  3. hexadecanoyl-CoA + glycine = N-hexadecanoylglycine + CoA + H(+)

  Catalysed by GATM, GLYATL3cytosol

• eicosanoyl-CoAReach from here →
  1. L-ornithine carboxy-lyase (putrescine-forming)
  2. acetyl-CoA:putrescine N-acetyltransferase
  3. long-chain-fatty-acid-CoA ligase (eicosanoate)

  Catalysed by ODC1, SAT1, SAT2, SLC27A2 and 7 morecytosolOne of 12 routes

• itaconyl-CoAReach from here →
  1. L-ornithine carboxy-lyase (putrescine-forming)
  2. acetyl-CoA:putrescine N-acetyltransferase
  3. Itaconate:CoA ligase (GDP-forming)

  Catalysed by ODC1, SAT1, SAT2, SUCLG1 and 2 morecytosol, mitochondrial matrixOne of 2 routes

• (9Z,12Z)-octadecadienoyl-CoAReach from here →
  1. L-ornithine carboxy-lyase (putrescine-forming)
  2. acetyl-CoA:putrescine N-acetyltransferase
  3. long-chain-fatty-acid-CoA ligase ((6Z,9Z)-octadecadienoic acid)

  Catalysed by ODC1, SAT1, SAT2, SLC27A2 and 7 morecytosolOne of 14 routes

• tetradecanoyl-CoAReach from here →
  1. L-ornithine carboxy-lyase (putrescine-forming)
  2. acetyl-CoA:putrescine N-acetyltransferase
  3. long-chain-fatty-acid-CoA ligase (myristic acid)

  Catalysed by ODC1, SAT1, SAT2, SLC27A2 and 7 morecytosolOne of 12 routes

• octanoyl-CoAReach from here →
  1. L-ornithine carboxy-lyase (putrescine-forming)
  2. acetyl-CoA:putrescine N-acetyltransferase
  3. Octanoyl-CoA:L-carnitine O-octanoyltransferase

  Catalysed by ODC1, SAT1, SAT2, CROTcytosol, mitochondrial matrixOne of 21 routes

• phytanoyl-CoAReach from here →
  1. L-ornithine carboxy-lyase (putrescine-forming)
  2. acetyl-CoA:putrescine N-acetyltransferase
  3. phytanate-CoA ligase

  Catalysed by ODC1, SAT1, SAT2cytosolOne of 5 routes

• octadecanoyl-CoAReach from here →
  1. L-ornithine carboxy-lyase (putrescine-forming)
  2. acetyl-CoA:putrescine N-acetyltransferase
  3. long-chain-fatty-acid-CoA ligase (stearate)

  Catalysed by ODC1, SAT1, SAT2, SLC27A2 and 6 morecytosolOne of 15 routes

• prostaglandin E1Reach from here →
  1. L-Arginine:glycine amidinotransferase
  2. gamma-L-glutamyl-L-cysteine:glycine ligase (ADP-forming)
  3. transport of GSH and prostaglandin E1 (cytosol to extracellular)

  Catalysed by GATM, GSS, SLCO2A1cytosol, extracellular spaceOne of 2 routes

• prostaglandin I2Reach from here →
  1. L-Arginine:glycine amidinotransferase
  2. gamma-L-glutamyl-L-cysteine:glycine ligase (ADP-forming)
  3. transport of GSH and prostaglandin I2 (cytosol to extracellular)

  Catalysed by GATM, GSS, SLCO2A1cytosol, extracellular spaceOne of 2 routes

• prostaglandin F2alphaReach from here →
  1. L-Arginine:glycine amidinotransferase
  2. gamma-L-glutamyl-L-cysteine:glycine ligase (ADP-forming)
  3. transport of GSH and prostaglandin F2alpha (cytosol to extracellular)

  Catalysed by GATM, GSS, SLCO2A1cytosol, extracellular spaceOne of 2 routes

• prostaglandin H2Reach from here →
  1. L-Arginine:glycine amidinotransferase
  2. gamma-L-glutamyl-L-cysteine:glycine ligase (ADP-forming)
  3. transport of GSH and prostaglandin H2 (cytosol to extracellular)

  Catalysed by GATM, GSS, SLCO2A1cytosol, extracellular spaceOne of 4 routes

• prostaglandin D2Reach from here →
  1. L-Arginine:glycine amidinotransferase
  2. gamma-L-glutamyl-L-cysteine:glycine ligase (ADP-forming)
  3. transport of GSH and prostaglandin D2 (cytosol to extracellular)

  Catalysed by GATM, GSS, SLCO2A1cytosol, extracellular spaceOne of 2 routes

• (15S)-hydroxy-(5Z,8Z,11Z,13E)-eicosatetraenoateReach from here →
  1. L-Arginine:glycine amidinotransferase
  2. gamma-L-glutamyl-L-cysteine:glycine ligase (ADP-forming)
  3. Glutathione: 15-HPETE oxidoreductase

  Catalysed by GATM, GSS, GPX5, GPX1 and 7 morecytosolOne of 3 routes

• S-adenosyl 3-(methylsulfanyl)propylamineReach from here →
  1. L-ornithine carboxy-lyase (putrescine-forming)
  2. S-adenosylmethioninamine:putrescine 3-aminopropyltransferase
  3. S-adenosyl 3-(methylsulfanyl)propylamine + putrescine = S-methyl-5'-thioadenosine + spermidine + H(+)

  Catalysed by ODC1, SRMcytosolOne of 2 routes

• 7,8-dihydrofolateReach from here →
  1. L-Arginine:glycine amidinotransferase
  2. 5,10-Methylenetetrahydrofolate:glycine hydroxymethyltransferase
  3. 5,6,7,8-tetrahydrofolate:NAD+ oxidoreductase

  Catalysed by GATM, SHMT1, DHFR, DHFR2cytosolOne of 4 routes

• 5-formimidoyltetrahydrofolateReach from here →
  1. L-Arginine:glycine amidinotransferase
  2. 5,10-Methylenetetrahydrofolate:glycine hydroxymethyltransferase
  3. 5-Formiminotetrahydrofolate:L-glutamate N-formiminotransferase

  Catalysed by GATM, SHMT1, FTCDcytosolOne of 2 routes

• leukotriene A4Reach from here →
  1. L-Arginine:glycine amidinotransferase
  2. gamma-L-glutamyl-L-cysteine:glycine ligase (ADP-forming)
  3. transport of GSH and leukotriene A4 (cytosol to extracellular)

  Catalysed by GATM, GSS, SLCO2A1cytosol, extracellular spaceOne of 3 routes

• (R)-S-lactoylglutathioneReach from here →
  1. L-Arginine:glycine amidinotransferase
  2. gamma-L-glutamyl-L-cysteine:glycine ligase (ADP-forming)
  3. (R)-S-Lactoylglutathione methylglyoxal-lyase (isomerizing)

  Catalysed by GATM, GSS, GLO1, GLOD4cytosolOne of 3 routes

• propane-1,3-diamineReach from here →
  1. L-ornithine carboxy-lyase (putrescine-forming)
  2. S-adenosylmethioninamine:putrescine 3-aminopropyltransferase
  3. [eIF5A protein]-L-lysine + spermidine = [eIF5A protein]-deoxyhypusine + propane-1,3-diamine

  Catalysed by ODC1, SRM, DHPScytosol

• 2-oxoadipateReach from here →
  1. L-Arginine:glycine amidinotransferase
  2. glycine + 2-oxoglutarate = glyoxylate + L-glutamate
  3. L-2-aminoadipate + glyoxylate = 2-oxoadipate + glycine

  Catalysed by GATM, AADATcytosol

• 3,5-diiodo-L-tyrosineReach from here →
  1. transport of ornithine and tyrosine (cytosol to extracellular)
  2. thyroid peroxidase (iodine)
  3. thyroid peroxidase (3-iodo-L-tyrosine)

  Catalysed by SLC7A6, TPOextracellular space, cytosolOne of 2 routes

• N-acetyl-D-glucosamine 6-phosphateReach from here →
  1. transport of glutamine and ornithine (cytosol to extracellular)
  2. L-glutamine:D-fructose-6-phosphate isomerase (deaminating)
  3. acetyl-CoA:D-glucosamine-6-phosphate N-acetyltransferase

  Catalysed by SLC7A6, GFPT2, GFPT1, GNPNAT1extracellular space, cytosolOne of 4 routes

• N(6)-(1,2-dicarboxyethyl)-AMPReach from here →
  1. transport of alanine and ornithine (cytosol to extracellular)
  2. oxaloacetate + L-alanine = L-aspartate + pyruvate
  3. IMP:L-aspartate ligase (GDP-forming)

  Catalysed by SLC7A6, AGXT2, ADSS2, ADSS1extracellular space, cytosolOne of 2 routes

• biotinReach from here →
  1. L-ornithine(out) + L-lysine(in) = L-ornithine(in) + L-lysine(out)
  2. biocytin + H2O = biotin + L-lysine
  3. Biotinidase (Biotin), Mitochondrial

  Catalysed by SLC25A2, SLC25A15, BTDinner side of the membrane, outer side of the membrane, mitochondrial matrixOne of 5 routes

• beta-D-fructose 6-phosphateReach from here →
  1. transport of glutamine and ornithine (cytosol to extracellular)
  2. L-glutamine:D-fructose-6-phosphate isomerase (deaminating)
  3. alpha-D-glucosamine 6-phosphate + H2O = beta-D-fructose 6-phosphate + NH4(+)

  Catalysed by SLC7A6, GFPT2, GFPT1, GNPDA1 and 1 moreextracellular space, cytosol

• S-formylglutathioneReach from here →
  1. L-Arginine:glycine amidinotransferase
  2. gamma-L-glutamyl-L-cysteine:glycine ligase (ADP-forming)
  3. alcohol dehydrogenase (formaldehyde)

  Catalysed by GATM, GSS, ADH1C, ADH1A and 4 morecytosolOne of 2 routes

• 2-methyl-3-oxopropanoateReach from here →
  1. transport of alanine and ornithine (cytosol to extracellular)
  2. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  3. (R)-3-amino-2-methylpropanoate + pyruvate = 2-methyl-3-oxopropanoate + L-alanine

  Catalysed by SLC7A6, AGXT2extracellular space, cytosol, mitochondrial matrix