L-ornithine

Three steps

• oxaloacetateReach from here →
  1. L-ornithine carboxy-lyase (putrescine-forming)
  2. acetyl-CoA:putrescine N-acetyltransferase
  3. ATP citrate lyase (citrate)

  Catalysed by ODC1, SAT1, SAT2, ACLYcytosolOne of 11 routes

• selaneReach from here →
  1. transport of alanine and ornithine (cytosol to extracellular)
  2. L-selenocysteine selenide-lyase (L-alanine-forming)
  3. L-selenocysteine selenide-lyase (L-alanine-forming)

  Catalysed by SLC7A6, SCLYextracellular space, cytosolOne of 3 routes

• anthranilateReach from here →
  1. transport of alanine and ornithine (cytosol to extracellular)
  2. L-kynurenine + H2O = anthranilate + L-alanine + H(+)
  3. L-kynurenine + H2O = anthranilate + L-alanine + H(+)

  Catalysed by SLC7A6, KYNUextracellular space, cytosol

• D-alanyl-D-alanineReach from here →
  1. L-Arginine:glycine amidinotransferase
  2. transport of D-alanine and glycine (cytosol to extracellular)
  3. Formation of Alanylalanine

  Catalysed by GATM, SLC3A2, SLC7A10cytosol, extracellular space

• (S)-lactateReach from here →
  1. transport of alanine and ornithine (cytosol to extracellular)
  2. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  3. (S)-lactate + NAD(+) = pyruvate + NADH + H(+)

  Catalysed by SLC7A6, AGXT2, LDHA, LDHB and 3 moreextracellular space, cytosol, mitochondrial matrixOne of 2 routes

• CHEBI:16668Reach from here →
  1. transport of cysteine and ornithine (cytosol to extracellular)
  2. L-Cysteine:oxygen oxidoreductase
  3. 3-sulfino-L-alanine carboxy-lyase

  Catalysed by SLC7A6, CDO1, GAD1, CSADextracellular space, cytosol

• 3'-deoxy-3',4'-didehydro-CTPReach 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. CTP + AH2 + S-adenosyl-L-methionine = 3'-deoxy-3',4'-didehydro-CTP + 5'-deoxyadenosine + L-methionine + A + H2O + H(+)

  Catalysed by SLC7A6, MOCOS, RSAD2extracellular space, cytosol

• cyanamideReach from here →
  1. L-Arginine:glycine amidinotransferase
  2. L-Arginine amidinohydrolase
  3. urea = cyanamide + H2O

  Catalysed by GATM, ARG1, CA1, CA2cytosol

• adenineReach from here →
  1. L-ornithine carboxy-lyase (putrescine-forming)
  2. S-adenosylmethioninamine:putrescine 3-aminopropyltransferase
  3. S-methyl-5'-thioadenosine:phosphate S-methyl-5-thio-alpha-D-ribosyl-transferase

  Catalysed by ODC1, SRM, MTAPcytosolOne of 2 routes

• pyridoxineReach from here →
  1. transport of alanine and ornithine (cytosol to extracellular)
  2. L-selenocysteine selenide-lyase (L-alanine-forming)
  3. pyridoxine-5'-phosphate phosphohydrolase

  Catalysed by SLC7A6, SCLY, PHOSPHO2, PDXPextracellular space, cytosolOne of 2 routes

• (S)-2-hydroxyglutarateReach 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. (S)-2-hydroxyglutarate + A = 2-oxoglutarate + AH2

  Catalysed by SLC7A6, MOCOS, L2HGDHextracellular space, cytosol

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

  Catalysed by SLC7A6, GOT2, TAT, MIFextracellular space, cytosol, mitochondrial matrix

• formaldehydeReach from here →
  1. L-Arginine:glycine amidinotransferase
  2. S-Adenosyl-L-methionine:glycine N-methyltransferase
  3. sarcosine:oxygen oxidoreductase (demethylating)

  Catalysed by GATM, GNMT, PIPOX, SARDHcytosol, peroxisomeOne of 5 routes

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

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

• pantetheine 4'-phosphateReach from here →
  1. transport of cysteine and ornithine (cytosol to extracellular)
  2. (R)-4'-phosphopantothenate:L-cysteine ligase
  3. N-[(R)-4'-Phosphopantothenoyl]-L-cysteine carboxy-lyase

  Catalysed by SLC7A6, PPCS, PPCDCextracellular space, cytosolOne of 2 routes

• trans-1,2-dithiane-4,5-diolReach from here →
  1. transport of alanine and ornithine (cytosol to extracellular)
  2. L-selenocysteine selenide-lyase (L-alanine-forming)
  3. selenocysteine lyase (dithiothreitol)

  Catalysed by SLC7A6, SCLYextracellular space, cytosol

• creatineReach from here →
  1. L-Arginine:glycine amidinotransferase
  2. L-Arginine:glycine amidinotransferase
  3. S-Adenosyl-L-methionine:guanidinoacetate N-methyltransferase

  Catalysed by GATM, GAMTcytosolOne of 2 routes

• N-acetyl-L-aspartateReach from here →
  1. transport of alanine and ornithine (cytosol to extracellular)
  2. oxaloacetate + L-alanine = L-aspartate + pyruvate
  3. acetyl-CoA:L-aspartate N-acetyltransferase

  Catalysed by SLC7A6, AGXT2, NAT8Lextracellular space, cytosol, mitochondrial matrixOne of 2 routes

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

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

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

  Catalysed by GATM, SLC6A14, SLC26A6cytosol, extracellular spaceOne of 3 routes

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

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

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

  Catalysed by SLC7A6, TPOextracellular space, cytosol

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

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

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

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

• CHEBI:17208Reach from here →
  1. L-Arginine:glycine amidinotransferase
  2. gamma-L-glutamyl-L-cysteine:glycine ligase (ADP-forming)
  3. glutathione:dehydroascorbate oxidoreductase

  Catalysed by GATM, GSScytosol

• (S)-4-hydroxymandelateReach from here →
  1. transport of ornithine and tyrosine (cytosol to extracellular)
  2. L-tyrosine:2-oxoglutarate aminotransferase
  3. 3-(4-hydroxyphenyl)pyruvate + O2 = (S)-4-hydroxymandelate + CO2

  Catalysed by SLC7A6, GOT2, GOT1, TAT and 1 moreextracellular space, cytosol

• homocysteineReach from here →
  1. L-Arginine:glycine amidinotransferase
  2. gamma-L-glutamyl-L-cysteine:glycine ligase (ADP-forming)
  3. Formation of Homocystine

  Catalysed by GATM, GSScytosol

• 17alpha-hydroxyprogesteroneReach from here →
  1. N2-Acetyl-L-ornithine amidohydrolase
  2. N2-Acetyl-L-ornithine amidohydrolase
  3. cytochrome P450 (17alpha-hydroxyprogesterone)

  Catalysed by ACY1, CYP17A1cytosol, endoplasmic reticulum

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

  Catalysed by ODC1, SAT1, SAT2, NAT2 and 1 morecytosol

• aceneuramateReach from here →
  1. transport of alanine and ornithine (cytosol to extracellular)
  2. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  3. aceneuramate = aldehydo-N-acetyl-D-mannosamine + pyruvate

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

• CHEBI:17312Reach from here →
  1. L-ornithine carboxy-lyase (putrescine-forming)
  2. acetyl-CoA:putrescine N-acetyltransferase
  3. N1,N12-diacetylspermine to N1-acetylspermine conversion

  Catalysed by ODC1, SAT1, SAT2cytosolOne of 2 routes

• 5'-deoxyadenosineReach from here →
  1. transport of alanine and ornithine (cytosol to extracellular)
  2. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  3. N(1)-methylguanosine(37) in tRNA(Phe) + pyruvate + S-adenosyl-L-methionine = 4-demethylwyosine(37) in tRNA(Phe) + 5'-deoxyadenosine + L-methionine + CO2 + H2O

  Catalysed by SLC7A6, AGXT2, TYW1B, TYW1extracellular space, cytosol, mitochondrial matrixOne of 5 routes

• all-trans-retinolReach 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. all-trans-13,14-dihydroretinol + A = all-trans-retinol + AH2

  Catalysed by SLC7A6, MOCOS, RETSATextracellular space, cytosol

• sulfiteReach from here →
  1. L-Arginine:glycine amidinotransferase
  2. gamma-L-glutamyl-L-cysteine + glycine + ATP = glutathione + ADP + phosphate + H(+)
  3. thiosulfate + 2 glutathione = glutathione disulfide + hydrogen sulfide + sulfite + 2 H(+)

  Catalysed by GATM, GSS, TSTD1cytosolOne of 2 routes

• diiodineReach from here →
  1. transport of ornithine and tyrosine (cytosol to extracellular)
  2. thyroid peroxidase (iodine)
  3. iodide:hydrogen-peroxide oxidoreductase

  Catalysed by SLC7A6, TPOextracellular space, cytosolOne of 2 routes

• nitrateReach from here →
  1. L-Arginine:glycine amidinotransferase
  2. L-Arginine, NADPH:Oxygen Oxidoreductase (Nitric-Oxide-Forming)
  3. Fe(II)-heme b-[protein] + nitric oxide + O2 = Fe(III)-heme b-[protein] + nitrate

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

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

  Catalysed by SLC7A6, MOCOS, DIO1extracellular space, cytosol

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

  Catalysed by SLC7A6, MOCOS, DIO1, DIO3extracellular space, cytosolOne of 2 routes

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

  Catalysed by SLC7A6, MOCOS, DIO1extracellular space, cytosolOne of 2 routes

• (7Z,10Z,13Z)-hexadecatrienoyl-CoAReach from here →
  1. L-ornithine carboxy-lyase (putrescine-forming)
  2. acetyl-CoA:putrescine N-acetyltransferase
  3. Fatty Acid Beta Oxidation (C18:3->C16:3), Mitochondrial

  Catalysed by ODC1, SAT1, SAT2, HADHA and 2 morecytosol, mitochondrial matrix

• CHEBI:17724Reach from here →
  1. L-Arginine:glycine amidinotransferase
  2. S-Adenosyl-L-methionine:glycine N-methyltransferase
  3. dimethylglycine dehydrogenase (formaldehyde)

  Catalysed by GATM, GNMT, PDPR, DMGDHcytosol

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

  Catalysed by GATM, SLC6A14, SLC6A12cytosol, extracellular spaceOne of 3 routes

• 4-methylumbelliferyl acetateReach from here →
  1. N2-Acetyl-L-ornithine amidohydrolase
  2. N2-Acetyl-L-ornithine amidohydrolase
  3. 4-methylumbelliferyl acetate + H2O = 4-methylumbelliferone + acetate + H(+)

  Catalysed by ACY1, CES2, ESD, CES1cytosol

• urateReach from here →
  1. L-Arginine:glycine amidinotransferase
  2. L-Arginine amidinohydrolase
  3. Peroxidase

  Catalysed by GATM, ARG1, MPOcytosol, nucleusOne of 4 routes

• methylarsonous acidReach from here →
  1. L-Arginine:glycine amidinotransferase
  2. gamma-L-glutamyl-L-cysteine + glycine + ATP = glutathione + ADP + phosphate + H(+)
  3. methylarsonate + 2 glutathione + H(+) = methylarsonous acid + glutathione disulfide + H2O

  Catalysed by GATM, GSS, GSTO1, GSTO2cytosol

• 3-oxopropanoic acidReach from here →
  1. transport of alanine and ornithine (cytosol to extracellular)
  2. beta-alanine(out) + L-alanine(in) = beta-alanine(in) + L-alanine(out)
  3. beta-alanine:2-oxoglutarate aminotransferase

  Catalysed by SLC7A6, SLC7A10, ABATextracellular space, cytosol, inner side of the membrane, outer side of the membrane, mitochondrial matrix

• fumaric acidReach from here →
  1. Amino Acid-Polyamine-Organocation (Apc) Tcdb:2.A.3.8.15
  2. L-Citrulline:L-aspartate ligase (AMP-forming)
  3. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)

  Catalysed by SLC7A9, ASS1, ASLextracellular space, cytosolOne of 2 routes

• thiocyanateReach from here →
  1. transport of cysteine and ornithine (cytosol to extracellular)
  2. L-Cysteine:2-oxoglutarate aminotransferase
  3. 3-mercaptopyruvate sulfurtransferase (hydrogen-cyanide)

  Catalysed by SLC7A6, GOT2, GOT1, MPST and 1 moreextracellular space, cytosol

• dTDPReach 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

• indole-3-acetaldehydeReach from here →
  1. transport of ornithine and tryptophan (cytosol to extracellular)
  2. L-tryptophan decarboxy-lyase
  3. Tryptamine:oxygen oxidoreductase(deaminating)

  Catalysed by SLC7A6, DDC, AOC1, MAOA and 1 moreextracellular space, cytosolOne of 2 routes