L-ornithine

Three steps

• N-(9Z-octadecenoyl)-(2S)-hydroxyglycineReach from here →
  1. L-Arginine:glycine amidinotransferase
  2. glycine + 2-oxoglutarate = glyoxylate + L-glutamate
  3. N-(9Z-octadecenoyl)-(2S)-hydroxyglycine = (9Z)-octadecenamide + glyoxylate

  Catalysed by GATM, AADAT, PAMcytosolOne of 2 routes

• N-(9Z,12Z,15Z)-octadecatrienoyl-(2S)-hydroxyglycineReach from here →
  1. L-Arginine:glycine amidinotransferase
  2. glycine + 2-oxoglutarate = glyoxylate + L-glutamate
  3. N-(9Z,12Z,15Z)-octadecatrienoyl-(2S)-hydroxyglycine = (9Z,12Z,15Z)-octadecatrienamide + glyoxylate

  Catalysed by GATM, AADAT, PAMcytosol

• 2-amino-2-deoxy-6-phospho-D-glucono-1,5-lactoneReach from here →
  1. transport of glutamine and ornithine (cytosol to extracellular)
  2. D-fructose 6-phosphate + L-glutamine = D-glucosamine 6-phosphate + L-glutamate
  3. D-glucosamine 6-phosphate + NADP(+) = 2-amino-2-deoxy-6-phospho-D-glucono-1,5-lactone + NADPH + 2 H(+)

  Catalysed by SLC7A6, GFPT2, GFPT1, H6PDextracellular space, cytosol

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

  Catalysed by GATM, DPEP1cytosol

• S-adenosylglutathioneReach from here →
  1. L-Arginine:glycine amidinotransferase
  2. gamma-L-glutamyl-L-cysteine + glycine + ATP = glutathione + ADP + phosphate + H(+)
  3. apo-[alkylcobalamin reductase] + adenosylcob(III)alamin + glutathione = S-adenosylglutathione + cob(I)alamin-[alkylcobalamin reductase] + H(+)

  Catalysed by GATM, GSS, MMACHCcytosol

• N(2)-formyl-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(+)
  3. N(1)-(5-phospho-beta-D-ribosyl)glycinamide + (6R)-10-formyltetrahydrofolate = N(2)-formyl-N(1)-(5-phospho-beta-D-ribosyl)glycinamide + (6S)-5,6,7,8-tetrahydrofolate + H(+)

  Catalysed by GATM, GARTcytosol

• kinetensin-(1-8)Reach from here →
  1. transport of ornithine and phenylalanine (cytosol to extracellular)
  2. Chymase
  3. kinetensin + H2O = kinetensin-(1-8) + L-leucine

  Catalysed by SLC7A6, CMA1, ACE2extracellular space, cytosol

• (7Z)-octadecenoyl-CoAReach from here →
  1. L-ornithine carboxy-lyase (putrescine-forming)
  2. acetyl-CoA:putrescine N-acetyltransferase
  3. carnitine O-palmitoyltransferase ((7Z)-octadecenoyl-CoA)

  Catalysed by ODC1, SAT1, SAT2, CPT1A and 2 morecytosolOne of 3 routes

• CHEBI:15345Reach from here →
  1. L-ornithine carboxy-lyase (putrescine-forming)
  2. acetyl-CoA:putrescine N-acetyltransferase
  3. Acetoacetate:CoA ligase (AMP-forming)

  Catalysed by ODC1, SAT1, SAT2, AACScytosolOne of 2 routes

• cholineReach from here →
  1. L-ornithine carboxy-lyase (putrescine-forming)
  2. acetyl-CoA:putrescine N-acetyltransferase
  3. Acetyl-CoA:choline O-acetyltransferase

  Catalysed by ODC1, SAT1, SAT2, CHATcytosol, nucleusOne of 2 routes

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

  Catalysed by GATM, GSS, SLCO1A2, SLCO1B3cytosol, extracellular spaceOne of 2 routes

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

  Catalysed by SLC7A6, AGXT2, FAHD1extracellular space, cytosol, mitochondrial matrixOne of 2 routes

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

  Catalysed by SLC7A6, AGXT2, SLC16A4, SLC16A5 and 4 moreextracellular space, cytosol, mitochondrial matrixOne of 4 routes

• CHEBI:15380Reach from here →
  1. L-ornithine carboxy-lyase (putrescine-forming)
  2. acetyl-CoA:putrescine N-acetyltransferase
  3. 5-aminolevulinate synthase (2-amino-3-oxoadipate)

  Catalysed by ODC1, SAT1, SAT2, ALAS1 and 1 morecytosol, mitochondrial matrixOne of 8 routes

• CHEBI:15477Reach from here →
  1. L-ornithine carboxy-lyase (putrescine-forming)
  2. acetyl-CoA:putrescine N-acetyltransferase
  3. glycine N-acyltransferase (2-methylbutyryl-CoA)

  Catalysed by ODC1, SAT1, SAT2, GLYAT and 2 morecytosol, mitochondrial matrixOne of 3 routes

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

  Catalysed by ODC1, SAT1, SAT2, CPT1A and 1 morecytosolOne of 2 routes

• isobutyryl-CoAReach from here →
  1. L-ornithine carboxy-lyase (putrescine-forming)
  2. acetyl-CoA:putrescine N-acetyltransferase
  3. acetyl-CoA C-acyltransferase (3-oxo-4-methyl-pentanoyl-CoA)

  Catalysed by ODC1, SAT1, SAT2, HADHA and 2 morecytosol, mitochondrial matrixOne of 3 routes

• 3-methylbut-2-enoyl-CoAReach from here →
  1. L-ornithine carboxy-lyase (putrescine-forming)
  2. acetyl-CoA:putrescine N-acetyltransferase
  3. glycine-N-acyltransferase (3-methylcrotonoylglycine)

  Catalysed by ODC1, SAT1, SAT2, GLYATcytosol, mitochondrial matrix

• isovaleryl-CoAReach from here →
  1. L-ornithine carboxy-lyase (putrescine-forming)
  2. acetyl-CoA:putrescine N-acetyltransferase
  3. glycine N-acyltransferase (isovalerylglycine)

  Catalysed by ODC1, SAT1, SAT2, GLYAT and 2 morecytosol, mitochondrial matrixOne of 3 routes

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

  Catalysed by ODC1, SAT1, SAT2, SLC27A2 and 7 morecytosolOne of 11 routes

• CHEBI:15519Reach from here →
  1. L-ornithine carboxy-lyase (putrescine-forming)
  2. acetyl-CoA:putrescine N-acetyltransferase
  3. propanoyl-CoA C-acyltransferase

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

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

  Catalysed by ODC1, SAT1, SAT2, SLC27A2 and 7 morecytosolOne of 11 routes

• oleoyl-CoAReach from here →
  1. L-ornithine carboxy-lyase (putrescine-forming)
  2. acetyl-CoA:putrescine N-acetyltransferase
  3. Transport of octadecenoyl-CoA into Mitochondrial Matrix

  Catalysed by ODC1, SAT1, SAT2, CPT1A and 2 morecytosolOne of 11 routes

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

  Catalysed by ODC1, SAT1, SAT2cytosolOne of 4 routes

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

  Catalysed by ODC1, SAT1, SAT2, ACSS3 and 2 morecytosolOne of 44 routes

• (S)-malateReach from here →
  1. L-Arginine:glycine amidinotransferase
  2. glycine:oxygen oxidoreductase (deaminating)
  3. acetyl-CoA[m] + glyoxalate[m] + H2O[m] = CoA[m] + H+[m] + malate[m]

  Catalysed by GATM, DAO, CLYBLcytosol, peroxisome, mitochondrial matrixOne of 4 routes

• (6R)-5,10-methylene-5,6,7,8-tetrahydrofolateReach from here →
  1. L-Arginine:glycine amidinotransferase
  2. (6R)-5,10-methylene-5,6,7,8-tetrahydrofolate + glycine + H2O = (6S)-5,6,7,8-tetrahydrofolate + L-serine
  3. (6R)-5,10-methylene-5,6,7,8-tetrahydrofolate + glycine + H2O = (6S)-5,6,7,8-tetrahydrofolate + L-serine

  Catalysed by GATM, SHMT1, SHMT2cytosolOne of 2 routes

• 10-formyltetrahydrofolic acidReach from here →
  1. L-Arginine:glycine amidinotransferase
  2. 5,10-Methylenetetrahydrofolate:glycine hydroxymethyltransferase
  3. Formate:tetrahydrofolate ligase (ADP-forming)

  Catalysed by GATM, SHMT1, MTHFD1cytosolOne of 3 routes

• 5-methyltetrahydrofolic acidReach from here →
  1. L-Arginine:glycine amidinotransferase
  2. 5,10-Methylenetetrahydrofolate:glycine hydroxymethyltransferase
  3. histamine N-methyltransferase (SAM)

  Catalysed by GATM, SHMT1, HNMTcytosol

• CHEBI:15650Reach from here →
  1. L-ornithine carboxy-lyase (putrescine-forming)
  2. acetyl-CoA:putrescine N-acetyltransferase
  3. N-acetyl-LTE4 to leukotriene E4 conversion

  Catalysed by ODC1, SAT1, SAT2, ZDHHC11B and 31 morecytosolOne of 6 routes

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

  Catalysed by SLC7A6, MOCOS, CHDHextracellular space, cytosol

• formateReach from here →
  1. transport of ornithine and tryptophan (cytosol to extracellular)
  2. L-tryptophan + O2 = N-formyl-L-kynurenine
  3. N-formyl-L-kynurenine + H2O = L-kynurenine + formate + H(+)

  Catalysed by SLC7A6, IDO1, TDO2, IDO2 and 1 moreextracellular space, cytosol

• CHEBI:15746Reach from here →
  1. L-ornithine carboxy-lyase (putrescine-forming)
  2. S-adenosylmethioninamine:putrescine 3-aminopropyltransferase
  3. S-adenosylmethioninamine:spermidine 3-aminopropyltransferase

  Catalysed by ODC1, SRM, SMScytosolOne of 3 routes

• 4-(2-amino-3-hydroxyphenyl)-2,4-dioxobutanoateReach from here →
  1. transport of ornithine and tryptophan (cytosol to extracellular)
  2. Transport of 3-Hydroxy-L-Kynurenine
  3. 3-hydroxy-L-kynurenine:2-oxoglutarate aminotransferase

  Catalysed by SLC7A6, SLC7A5, KYAT1, KYAT3 and 1 moreextracellular space, cytosol

• 3-hydroxyanthranilic acidReach from here →
  1. transport of ornithine and tryptophan (cytosol to extracellular)
  2. Transport of 3-Hydroxy-L-Kynurenine
  3. 3-Hydroxy-L-kynurenine hydrolase

  Catalysed by SLC7A6, SLC7A5, KYNUextracellular space, cytosol

• bromideReach from here →
  1. L-Arginine:glycine amidinotransferase
  2. gamma-L-glutamyl-L-cysteine:glycine ligase (ADP-forming)
  3. 2-bromoacetaldehyde:glutathione S-(formylmethyl)transferase

  Catalysed by GATM, GSS, MGST3, GSTA4 and 15 morecytosolOne of 2 routes

• N-carbamoyl-L-aspartic acidReach from here →
  1. Amino Acid-Polyamine-Organocation (Apc) Tcdb:2.A.3.8.15
  2. Carbamoyl-phosphate:L-ornithine carbamoyltransferase
  3. carbamoyl-phosphate:L-aspartate carbamoyltransferase

  Catalysed by SLC7A9, OTC, CADextracellular space, cytosol, mitochondrial matrixOne of 2 routes

• keto-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. D-glucosamine-6-phosphate aminohydrolase (ketol isomerizing)

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

• thioredoxin dithiolReach from here →
  1. L-Arginine:glycine amidinotransferase
  2. glycine:tRNA(Gly) ligase (AMP-forming)
  3. glycyl-tRNA(gly) phosphorylation

  Catalysed by GATM, GARS1cytosolOne of 18 routes

• methanethiolReach from here →
  1. transport of cysteine and ornithine (cytosol to extracellular)
  2. L-cysteine hydrogen-sulfide-lyase (deaminating
  3. methanethiol + O2 + H2O = hydrogen sulfide + formaldehyde + H2O2 + H(+)

  Catalysed by SLC7A6, CTH, SELENBP1extracellular space, cytosol

• a halide anionReach from here →
  1. L-Arginine:glycine amidinotransferase
  2. gamma-L-glutamyl-L-cysteine + glycine + ATP = glutathione + ADP + phosphate + H(+)
  3. RX + glutathione = an S-substituted glutathione + a halide anion + H(+)

  Catalysed by GATM, GSS, GSTT4, GSTA4 and 21 morecytosol

• CHEBI:16132Reach from here →
  1. transport of ornithine and threonine (cytosol to extracellular)
  2. L-threonine acetaldehyde-lyase (glycine-forming)
  3. deoxyribose-phosphate aldolase

  Catalysed by SLC7A6, DERAextracellular space, cytosol

• homogentisateReach from here →
  1. transport of ornithine and tyrosine (cytosol to extracellular)
  2. L-tyrosine:2-oxoglutarate aminotransferase
  3. 4-Hydroxyphenylpyruvate:oxygen oxidoreductase (hydroxylating,decarboxylating)

  Catalysed by SLC7A6, GOT2, GOT1, TAT and 1 moreextracellular space, cytosolOne of 2 routes

• sulfateReach from here →
  1. L-Arginine:glycine amidinotransferase
  2. transport of arginine and chloride (cytosol to extracellular)
  3. transport of chloride and sulfate (cytosol to extracellular)

  Catalysed by GATM, SLC6A14, SLC26A6cytosol, extracellular spaceOne of 4 routes

• 2-methyl-3-oxopropanoic acidReach from here →
  1. transport of alanine and ornithine (cytosol to extracellular)
  2. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  3. (S)-3-amino-2-methylpropionate transaminase (2-methyl-3-oxopropanoate)

  Catalysed by SLC7A6, AGXT2, ABATextracellular space, cytosol, mitochondrial matrixOne of 3 routes

• nitriteReach from here →
  1. L-Arginine:glycine amidinotransferase
  2. L-Arginine, NADPH:Oxygen Oxidoreductase (Nitric-Oxide-Forming)
  3. 4 nitric oxide + O2 + 2 H2O = 4 nitrite + 4 H(+)

  Catalysed by GATM, NOS3, NOS1, NOS2 and 1 morecytosol

• 11-cis-retinolReach from here →
  1. L-ornithine carboxy-lyase (putrescine-forming)
  2. acetyl-CoA:putrescine N-acetyltransferase
  3. Palmitoyl-CoA:retinol O-acyltransferase

  Catalysed by ODC1, SAT1, SAT2, DGAT1 and 4 morecytosol

• cob(II)alaminReach 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. 2 cob(II)alamin + A + 2 H2O + 2 H(+) = 2 aquacob(III)alamin + AH2

  Catalysed by SLC7A6, MOCOS, MTRRextracellular space, cytosol

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

  Catalysed by GATM, GSS, SLCO1A2, SLCO1B3cytosol, extracellular spaceOne of 2 routes

• 2-phenylacetaldehydeReach from here →
  1. transport of ornithine and phenylalanine (cytosol to extracellular)
  2. L-phenylalanine carboxy-lyase (phenylethylamine-forming)
  3. Phenethylamine:oxygen oxidoreductase (deaminating)

  Catalysed by SLC7A6, DDC, AOC2, AOC1 and 3 moreextracellular space, cytosolOne of 2 routes