L-ornithine

Two steps

• N(1)-(5-phospho-beta-D-ribosyl)glycinamideReach from here →
  1. L-Arginine:glycine amidinotransferase
  2. 5-phospho-beta-D-ribosylamine + glycine + ATP = N(1)-(5-phospho-beta-D-ribosyl)glycinamide + ADP + phosphate + H(+)

  Catalysed by GATM, GARTcytosol

• L-cystine-bis-glycineReach from here →
  1. L-Arginine:glycine amidinotransferase
  2. L-cystine-bis-glycine + 2 H2O = L-cystine + 2 glycine

  Catalysed by GATM, DPEP1, DPEP2cytosol

• glycyldehydrophenylalanineReach from here →
  1. L-Arginine:glycine amidinotransferase
  2. glycyldehydrophenylalanine + H2O = 2,3-didehydrophenylalanine + glycine

  Catalysed by GATM, DPEP1cytosol

• 2-formamido-N(1)-(5-O-phospho-beta-D-ribosyl)acetamidineReach from here →
  1. transport of glutamine and ornithine (cytosol to extracellular)
  2. N(2)-formyl-N(1)-(5-phospho-beta-D-ribosyl)glycinamide + L-glutamine + ATP + H2O = 2-formamido-N(1)-(5-O-phospho-beta-D-ribosyl)acetamidine + L-glutamate + ADP + phosphate + H(+)

  Catalysed by SLC7A6, PFASextracellular space, cytosol

• kinetensinReach from here →
  1. transport of ornithine and phenylalanine (cytosol to extracellular)
  2. Chymase

  Catalysed by SLC7A6, CMA1extracellular space, cytosolOne of 3 routes

• an N-(fatty acyl)-glycineReach from here →
  1. L-Arginine:glycine amidinotransferase
  2. a fatty acyl-CoA + glycine = an N-(fatty acyl)-glycine + CoA + H(+)

  Catalysed by GATM, GLYATL2cytosol

• N-(9Z,12Z-octadecadienoyl)-glycineReach from here →
  1. L-Arginine:glycine amidinotransferase
  2. (9Z,12Z)-octadecadienoyl-CoA + glycine = N-(9Z,12Z-octadecadienoyl)-glycine + CoA + H(+)

  Catalysed by GATM, GLYATL2cytosol

• acetaldehydeReach from here →
  1. transport of ornithine and threonine (cytosol to extracellular)
  2. L-threonine acetaldehyde-lyase (glycine-forming)

  Catalysed by SLC7A6extracellular space, cytosol

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

  Catalysed by SLC7A6, AGXT2extracellular space, cytosol, mitochondrial matrixOne of 17 routes

• 4a-hydroxytetrahydrobiopterinReach from here →
  1. transport of ornithine and tryptophan (cytosol to extracellular)
  2. L-tryptophan,tetrahydrobiopterin:oxygen oxidoreductase(5-hydroxylating)

  Catalysed by SLC7A6, TPH1, TPH2extracellular space, cytosolOne of 3 routes

• CHEBI:15570Reach from here →
  1. L-Arginine:glycine amidinotransferase
  2. transport of D-alanine and glycine (cytosol to extracellular)

  Catalysed by GATM, SLC3A2, SLC7A10cytosol, extracellular spaceOne of 5 routes

• CHEBI:15611Reach from here →
  1. L-Arginine:glycine amidinotransferase
  2. S-Adenosyl-L-methionine:glycine N-methyltransferase

  Catalysed by GATM, GNMTcytosol

• (4aS,6R)-4a-hydroxy-L-erythro-5,6,7,8-tetrahydrobiopterinReach from here →
  1. transport of ornithine and tryptophan (cytosol to extracellular)
  2. (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 SLC7A6, TPH1, TPH2extracellular space, cytosolOne of 2 routes

• trimethylenediamineReach from here →
  1. transport of lysine and ornithine (cytosol to extracellular)
  2. deoxyhypusine synthase (1,3-diaminopropane)

  Catalysed by SLC7A5, SLC7A6, DHPSextracellular space, cytosol

• CHEBI:15727Reach from here →
  1. transport of histidine and ornithine (cytosol to extracellular)
  2. L-histidine:beta-alanine ligase (ADP-forming)

  Catalysed by SLC7A5, SLC7A6, CARNS1extracellular space, cytosol

• CHEBI:15765Reach from here →
  1. transport of ornithine and tyrosine (cytosol to extracellular)
  2. L-Tyrosine,tetrahydrobiopterin:oxygen oxidoreductase (3-hydroxylating)

  Catalysed by SLC7A6, THextracellular space, cytosolOne of 5 routes

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

  Catalysed by SLC7A6, GFPT2, GFPT1extracellular space, cytosol

• ureaReach from here →
  1. L-Arginine:glycine amidinotransferase
  2. L-Arginine amidinohydrolase

  Catalysed by GATM, ARG1cytosolOne of 3 routes

• L-cystineReach from here →
  1. L-Arginine:glycine amidinotransferase
  2. L-cystine(out) + L-arginine(in) = L-cystine(in) + L-arginine(out)

  Catalysed by GATM, SLC7A9cytosol, inner side of the membrane, outer side of the membraneOne of 7 routes

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

  Catalysed by SLC7A6, AGXT2extracellular space, cytosol, mitochondrial matrix

• iodideReach from here →
  1. transport of ornithine and tyrosine (cytosol to extracellular)
  2. thyroid peroxidase (iodine)

  Catalysed by SLC7A6, TPOextracellular space, cytosolOne of 2 routes

• nitric oxideReach from here →
  1. L-Arginine:glycine amidinotransferase
  2. L-Arginine, NADPH:Oxygen Oxidoreductase (Nitric-Oxide-Forming)

  Catalysed by GATM, NOS3, NOS1, NOS2cytosolOne of 2 routes

• CHEBI:16523Reach from here →
  1. L-Arginine:glycine amidinotransferase
  2. transport of D-serine and glycine (cytosol to extracellular)

  Catalysed by GATM, SLC3A2, SLC7A10cytosol, extracellular spaceOne of 4 routes

• protein polypeptide chainReach from here →
  1. L-Arginine:glycine amidinotransferase
  2. PROTEIN_BS

  Catalysed by GATMcytosolOne of 17 routes

• 2-phenylacetamideReach from here →
  1. transport of ornithine and phenylalanine (cytosol to extracellular)
  2. L-phenylalanine:oxygen 2-oxidoreductase (decarboxylating)

  Catalysed by SLC7A6, EPXextracellular space, cytosol

• CHEBI:16633Reach from here →
  1. transport of alanine and ornithine (cytosol to extracellular)
  2. L-selenocysteine selenide-lyase (L-alanine-forming)

  Catalysed by SLC7A6, SCLYextracellular space, cytosol

• Met-tRNA(Met)Reach from here →
  1. transport of methionine and ornithine (cytosol to extracellular)
  2. L-Methionine:tRNAMet ligase (AMP-forming)

  Catalysed by SLC7A6, MARS1extracellular space, cytosol

• 4-methylsulfanyl-2-oxobutanoateReach from here →
  1. transport of methionine and ornithine (cytosol to extracellular)
  2. glyoxylate + L-methionine = 4-methylsulfanyl-2-oxobutanoate + glycine

  Catalysed by SLC7A6, AADATextracellular space, cytosol

• N-[(S)-lactoyl]-L-phenylalanineReach from here →
  1. transport of ornithine and phenylalanine (cytosol to extracellular)
  2. (S)-lactate + L-phenylalanine = N-[(S)-lactoyl]-L-phenylalanine + H2O

  Catalysed by SLC7A6, CNDP2extracellular space, cytosol

• 2-oxobutanoateReach from here →
  1. transport of ornithine and threonine (cytosol to extracellular)
  2. L-threonine ammonia-lyase (2-oxobutanoate-forming)

  Catalysed by SLC7A6, SDS, SDSLextracellular space, cytosol

• tryptamineReach from here →
  1. transport of ornithine and tryptophan (cytosol to extracellular)
  2. L-tryptophan decarboxy-lyase

  Catalysed by SLC7A6, DDCextracellular space, cytosol

• 2-oxoglutaramateReach from here →
  1. transport of glutamine and ornithine (cytosol to extracellular)
  2. L-Glutamine:pyruvate aminotransferase

  Catalysed by SLC7A6, KYAT1extracellular space, cytosolOne of 3 routes

• CHEBI:16856Reach from here →
  1. L-Arginine:glycine amidinotransferase
  2. gamma-L-glutamyl-L-cysteine:glycine ligase (ADP-forming)

  Catalysed by GATM, GSScytosolOne of 2 routes

• glyoxylic acidReach from here →
  1. L-Arginine:glycine amidinotransferase
  2. glycine:oxygen oxidoreductase (deaminating)

  Catalysed by GATM, DAOcytosol, peroxisome

• CHEBI:17380Reach from here →
  1. transport of ornithine and tryptophan (cytosol to extracellular)
  2. Transport of 3-Hydroxy-L-Kynurenine

  Catalysed by SLC7A6, SLC7A5extracellular space, cytosolOne of 2 routes

• (S)-1-pyrroline-5-carboxylateReach from here →
  1. L-Ornithine:2-oxo-acid aminotransferase
  2. aldehyde dehydrogenase (1-pyrroline-5-carboxylate)

  Catalysed by OAT, ALDH4A1mitochondrial matrix, cytosolOne of 4 routes

• agmatineReach from here →
  1. L-Arginine:glycine amidinotransferase
  2. L-arginine carboxy-lyase (agmatine-forming)

  Catalysed by GATMcytosol, mitochondrial matrix

• AH2Reach from here →
  1. transport of alanine and ornithine (cytosol to extracellular)
  2. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O

  Catalysed by SLC7A6, MOCOSextracellular space, cytosolOne of 4 routes

• S-methyl-5'-thioadenosineReach from here →
  1. L-ornithine carboxy-lyase (putrescine-forming)
  2. S-adenosylmethioninamine:putrescine 3-aminopropyltransferase

  Catalysed by ODC1, SRMcytosolOne of 2 routes

• 5-aminolevulinic acidReach from here →
  1. L-Arginine:glycine amidinotransferase
  2. succinyl-CoA:glycine C-succinyltransferase (decarboxylating)

  Catalysed by GATM, ALAS1, ALAS2, CPNE7cytosol, mitochondrial matrix

• indole-3-pyruvateReach from here →
  1. transport of ornithine and tryptophan (cytosol to extracellular)
  2. L-tryptophan + O2 + H2O = indole-3-pyruvate + H2O2 + NH4(+)

  Catalysed by SLC7A6, IL4I1extracellular space, cytosolOne of 2 routes

• Ala-tRNA(Ala)Reach from here →
  1. transport of alanine and ornithine (cytosol to extracellular)
  2. L-Alanine:tRNA(Ala) ligase (AMP-forming)

  Catalysed by SLC7A6, AARS1extracellular space, cytosol

• CHEBI:17769Reach from here →
  1. L-ornithine carboxy-lyase (putrescine-forming)
  2. Putrescine:oxygen oxidoreductase (deaminating)

  Catalysed by ODC1, AOC2, AOC1, AOC3cytosol, extracellular spaceOne of 2 routes

• trans-urocanateReach from here →
  1. transport of histidine and ornithine (cytosol to extracellular)
  2. L-histidine ammonia-lyase (urocanate-forming)

  Catalysed by SLC7A5, SLC7A6, HALextracellular space, cytosolOne of 2 routes

• 5-hydroxy-L-tryptophanReach from here →
  1. transport of ornithine and tryptophan (cytosol to extracellular)
  2. (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 SLC7A6, TPH1, TPH2extracellular space, cytosol

• 4-methyl-2-oxopentanoateReach from here →
  1. transport of leucine and ornithine (cytosol to extracellular)
  2. L-Leucine:2-oxoglutarate aminotransferase

  Catalysed by SLC7A6, BCAT2extracellular space, cytosol, mitochondrial matrixOne of 4 routes

• CHEBI:17927Reach from here →
  1. L-Arginine:glycine amidinotransferase
  2. Transport by Y+Lat-2 into Extracellular Space

  Catalysed by GATM, SLC3A2, SLC7A6cytosol, extracellular space

• 3-phenylpyruvateReach from here →
  1. transport of ornithine and phenylalanine (cytosol to extracellular)
  2. Phenylalanine Transaminase, Mitochondrial

  Catalysed by SLC7A6, GOT2, TATextracellular space, cytosol, mitochondrial matrixOne of 5 routes

• CHEBI:18095Reach from here →
  1. transport of alanine and ornithine (cytosol to extracellular)
  2. Transport of Hydroxy-Proline

  Catalysed by SLC7A6, SLC1A4extracellular space, cytosolOne of 4 routes

• N(1)-(5-phospho-D-ribosyl)glycinamideReach from here →
  1. L-Arginine:glycine amidinotransferase
  2. 5-Phospho-D-ribosylamine:glycine ligase (ADP-forming)

  Catalysed by GATM, GARTcytosol