L-ornithine

Three steps

• selenomethionineReach from here →
  1. transport of ornithine and threonine (cytosol to extracellular)
  2. L-threonine ammonia-lyase (2-oxobutanoate-forming)
  3. Selenomethionine methanethiol-lyase (deaminating)

  Catalysed by SLC7A6, SDS, SDSL, CTHextracellular space, cytosol

• CHEBI:27760Reach from here →
  1. transport of alanine and ornithine (cytosol to extracellular)
  2. L-selenocysteine selenide-lyase (L-alanine-forming)
  3. Selenocystathionine Lysteine-lyase (deaminating)

  Catalysed by SLC7A6, SCLY, CTHextracellular space, cytosolOne of 2 routes

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

  Catalysed by ODC1, SAT1, SAT2, SLC27A2 and 7 morecytosolOne of 5 routes

• 5(S)-HETEReach from here →
  1. L-Arginine:glycine amidinotransferase
  2. gamma-L-glutamyl-L-cysteine:glycine ligase (ADP-forming)
  3. Glutathione: 5-HPETE oxidoreductase

  Catalysed by GATM, GSS, GPX5, GPX1 and 7 morecytosolOne of 2 routes

• (2-hydroxyphenyl)acetic acidReach from here →
  1. transport of ornithine and phenylalanine (cytosol to extracellular)
  2. Phenylalanine Transaminase, Mitochondrial
  3. Phenylpyruvate:oxygen oxidoreductase (hydroxylating,decarboxylating)

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

• 3-mercaptolactic acidReach from here →
  1. transport of cysteine and ornithine (cytosol to extracellular)
  2. L-Cysteine:2-oxoglutarate aminotransferase
  3. 3-Mercaptolactate:NAD+ oxidoreductase

  Catalysed by SLC7A6, GOT2, GOT1, LDHB and 3 moreextracellular space, cytosol

• 2-oxopentanoateReach from here →
  1. N(omega),N(omega)-dimethyl-L-arginine(in) + L-ornithine(out) = N(omega),N(omega)-dimethyl-L-arginine(out) + L-ornithine(in)
  2. N(omega),N(omega)-dimethyl-L-arginine + pyruvate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + L-alanine
  3. 2-oxopentanoate + N(omega),N(omega)-dimethyl-L-arginine = 5-(3,3-dimethylguanidino)-2-oxopentanoate + L-2-aminopentanoate

  Catalysed by SLC25A2, AGXT2inner side of the membrane, outer side of the membraneOne of 2 routes

• CHEBI:28701Reach from here →
  1. L-ornithine carboxy-lyase (putrescine-forming)
  2. acetyl-CoA:putrescine N-acetyltransferase
  3. Propanoyl-CoA:acetyl-CoA C-acyltransferase

  Catalysed by ODC1, SAT1, SAT2, ACAA1 and 2 morecytosol, peroxisomeOne of 7 routes

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

  Catalysed by ACY1, CYP17A1cytosol, endoplasmic reticulum

• 3-disulfanyl-L-alanineReach from here →
  1. L-Arginine:glycine amidinotransferase
  2. L-cystine(out) + L-arginine(in) = L-cystine(in) + L-arginine(out)
  3. L-cystine thiocysteine-lyase (deaminating

  Catalysed by GATM, SLC7A9, CTHcytosol, inner side of the membrane, outer side of the membrane

• a dicarboxylateReach from here →
  1. L-Arginine:glycine amidinotransferase
  2. Hippurate (Benzoylglycinate) Formation
  3. N-benzoylglycine(out) + a dicarboxylate(in) = N-benzoylglycine(in) + a dicarboxylate(out)

  Catalysed by GATM, SLC22A6, SLC22A8cytosol, mitochondrial matrix, inner side of the membrane, outer side of the membraneOne of 2 routes

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

  Catalysed by GATM, GSS, CLIC1, GSTO1 and 1 morecytosol

• Ca(2+)Reach from here →
  1. transport of histidine and ornithine (cytosol to extracellular)
  2. Formation of homocarnosine
  3. Transport of Homocarnosine

  Catalysed by SLC7A5, SLC7A6, CARNS1extracellular space, cytosolOne of 4 routes

• 3-methyl-1-hydroxybutylthiamine diphosphateReach from here →
  1. transport of leucine and ornithine (cytosol to extracellular)
  2. L-Leucine:2-oxoglutarate aminotransferase
  3. branched chain keto acid dehydrogenase E1 (4-methyl-2-oxopentanoate)

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

• Asp-tRNA(Asp)Reach from here →
  1. transport of alanine and ornithine (cytosol to extracellular)
  2. oxaloacetate + L-alanine = L-aspartate + pyruvate
  3. L-Aspartate:tRNA(Asp) ligase (AMP-forming)

  Catalysed by SLC7A6, AGXT2, DARS1extracellular space, cytosol

• hypoioditeReach from here →
  1. transport of ornithine and tyrosine (cytosol to extracellular)
  2. thyroid peroxidase (iodine)
  3. iodide + H2O2 = hypoiodite + H2O

  Catalysed by SLC7A6, TPO, MPO, LPOextracellular space, cytosol

• a thiolReach from here →
  1. transport of cysteine and ornithine (cytosol to extracellular)
  2. L-cysteine hydrogen-sulfide-lyase (deaminating
  3. L-cysteinyl-[sulfatase] + 2 a thiol + O2 = an organic disulfide + 3-oxo-L-alanyl-[sulfatase] + hydrogen sulfide + H2O + H(+)

  Catalysed by SLC7A6, CTH, SUMF1extracellular space, cytosol

• hydrogenselenideReach 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. L-selenocysteine + AH2 = hydrogenselenide + L-alanine + A + H(+)

  Catalysed by SLC7A6, MOCOS, SCLYextracellular space, cytosol

• cholateReach from here →
  1. L-Arginine:glycine amidinotransferase
  2. gamma-L-glutamyl-L-cysteine:glycine ligase (ADP-forming)
  3. solute carrier organic anion transporter (cholate)

  Catalysed by GATM, GSS, SLCO1A2, SLCO1B3cytosol, extracellular spaceOne of 3 routes

• glycolateReach from here →
  1. L-Arginine:glycine amidinotransferase
  2. glycine:oxygen oxidoreductase (deaminating)
  3. glycolate:NADP+ oxidoreductase

  Catalysed by GATM, DAO, GRHPRcytosol, peroxisomeOne of 3 routes

• D-aspartateReach from here →
  1. transport of cysteine and ornithine (cytosol to extracellular)
  2. K(+)(in) + L-cysteine(out) + 3 Na(+)(out) + H(+)(out) = K(+)(out) + L-cysteine(in) + 3 Na(+)(in) + H(+)(in)
  3. D-aspartate(out) + K(+)(in) + 3 Na(+)(out) + H(+)(out) = D-aspartate(in) + K(+)(out) + 3 Na(+)(in) + H(+)(in)

  Catalysed by SLC7A6, SLC1A1, SLC1A7, SLC1A3 and 2 moreextracellular space, cytosol, inner side of the membrane, outer side of the membraneOne of 2 routes

• 3-acetamidopropanalReach from here →
  1. L-ornithine carboxy-lyase (putrescine-forming)
  2. N(1)-acetylspermidine + O2 + H2O = 3-acetamidopropanal + putrescine + H2O2
  3. N(1)-acetylspermidine + O2 + H2O = 3-acetamidopropanal + putrescine + H2O2

  Catalysed by ODC1, PAOXcytosolOne of 3 routes

• oxalateReach from here →
  1. L-Arginine:glycine amidinotransferase
  2. glycine + 2-oxoglutarate = glyoxylate + L-glutamate
  3. glyoxylate + O2 + H2O = oxalate + H2O2 + H(+)

  Catalysed by GATM, AADAT, HAO1cytosol

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

  Catalysed by GATM, SLC6A14, SLC26A4cytosol, extracellular spaceOne of 7 routes

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

  Catalysed by SLC7A6, KYNUextracellular space, cytosol

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

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

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

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

• malonic acidReach from here →
  1. L-Arginine:glycine amidinotransferase
  2. transport of arginine and chloride (cytosol to extracellular)
  3. Malonate Transport, Antiport with Chloride

  Catalysed by GATM, SLC6A14cytosol, extracellular spaceOne of 2 routes

• (9Z)-octadecenoateReach from here →
  1. L-Arginine:glycine amidinotransferase
  2. (9Z)-octadecenoyl-CoA + glycine = N-(9Z-octadecenoyl)glycine + CoA + H(+)
  3. (9Z)-octadecenoate + glycine = N-(9Z-octadecenoyl)glycine + H2O

  Catalysed by GATM, GLYATL3, GLYATL2, FAAH and 1 morecytosolOne of 3 routes

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

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

• an alcoholReach from here →
  1. L-Arginine:glycine amidinotransferase
  2. gamma-L-glutamyl-L-cysteine + glycine + ATP = glutathione + ADP + phosphate + H(+)
  3. a hydroperoxide + 2 glutathione = an alcohol + glutathione disulfide + H2O

  Catalysed by GATM, GSS, CP, PRDX6cytosol

• glutarateReach from here →
  1. L-Arginine:glycine amidinotransferase
  2. bile acid-CoA: amino acid N-acyltransferase
  3. glycocholate(out) + glutarate(in) = glycocholate(in) + glutarate(out)

  Catalysed by GATM, BAAT, SLC22A24cytosol, peroxisome, inner side of the membrane, outer side of the membraneOne of 2 routes

• (5Z,8Z,11Z,14Z)-eicosatetraenoateReach from here →
  1. L-Arginine:glycine amidinotransferase
  2. gamma-L-glutamyl-L-cysteine:glycine ligase (ADP-forming)
  3. transport of arachidonate and GSH (cytosol to extracellular)

  Catalysed by GATM, GSScytosol, extracellular spaceOne of 4 routes

• N-carbamoyl-L-aspartateReach 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 = N-carbamoyl-L-aspartate + phosphate + H(+)

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

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

  Catalysed by SLC7A6, AGXT2extracellular space, cytosol, mitochondrial matrixOne of 2 routes

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

  Catalysed by SLC7A6, GOT2, GOT1, MPST and 1 moreextracellular space, cytosolOne of 4 routes

• tetra-mu3-sulfido-tetrairon(2+)Reach from here →
  1. transport of cysteine and ornithine (cytosol to extracellular)
  2. De novo synthesis of 2Fe2S
  3. Synthesis of 4Fe4S from 2Fe2S

  Catalysed by SLC7A6, NDUFAB1, FDXR, FXN and 10 moreextracellular space, cytosol, mitochondrial matrix

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

  Catalysed by GATM, GSS, SLCO1A2, SLCO1B3cytosol, extracellular spaceOne of 5 routes

• 2-oxohexanoateReach from here →
  1. N(omega),N(omega)-dimethyl-L-arginine(in) + L-ornithine(out) = N(omega),N(omega)-dimethyl-L-arginine(out) + L-ornithine(in)
  2. N(omega),N(omega)-dimethyl-L-arginine + pyruvate = 5-(3,3-dimethylguanidino)-2-oxopentanoate + L-alanine
  3. 2-oxohexanoate + N(omega),N(omega)-dimethyl-L-arginine = L-2-aminohexanoate + 5-(3,3-dimethylguanidino)-2-oxopentanoate

  Catalysed by SLC25A2, AGXT2inner side of the membrane, outer side of the membraneOne of 2 routes

• L-alpha-aminobutyric acidReach from here →
  1. transport of ornithine and threonine (cytosol to extracellular)
  2. L-threonine ammonia-lyase (2-oxobutanoate-forming)
  3. glutamic-pyruvic transaminase ((S)-2-aminobutanoate)

  Catalysed by SLC7A6, SDS, SDSL, GPTextracellular space, cytosol

• taurocholateReach from here →
  1. L-Arginine:glycine amidinotransferase
  2. gamma-L-glutamyl-L-cysteine:glycine ligase (ADP-forming)
  3. solute carrier organic anion transporter (GSH)

  Catalysed by GATM, GSS, SLCO1A2, SLCO1B3cytosol, extracellular spaceOne of 3 routes

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

  Catalysed by SLC7A6, KYNUextracellular space, cytosol

• N-formylanthranilic acidReach from here →
  1. transport of ornithine and tryptophan (cytosol to extracellular)
  2. L-tryptophan:oxygen 2,3-oxidoreductase (decyclizing)
  3. Formylkynurenine hydrolase

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

• 3-hydroxyisovaleric acidReach from here →
  1. transport of leucine and ornithine (cytosol to extracellular)
  2. L-Leucine:2-oxoglutarate aminotransferase
  3. 4-methyl-2-oxopentanoate decarboxylation

  Catalysed by SLC7A6, BCAT2, ALOX12B, ALOXE3extracellular space, cytosol, mitochondrial matrix

• D-glucopyranose 6-phosphateReach from here →
  1. Amino Acid-Polyamine-Organocation (Apc) Tcdb:2.A.3.8.15
  2. Carbamoyl-phosphate:L-ornithine carbamoyltransferase
  3. glucose-6-phosphatase (carbamoyl-phosphate)

  Catalysed by SLC7A9, OTC, G6PC2extracellular space, cytosol, mitochondrial matrix, endoplasmic reticulum

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

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

• (S)-lactic acidReach from here →
  1. transport of alanine and ornithine (cytosol to extracellular)
  2. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  3. (S)-Lactate:NAD+ oxidoreductase

  Catalysed by SLC7A6, AGXT2, LDHB, LDHAL6Bextracellular space, cytosol, mitochondrial matrixOne of 7 routes

• 25-hydroxycholesterolReach 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. cholesterol + AH2 + O2 = 25-hydroxycholesterol + A + H2O

  Catalysed by SLC7A6, MOCOS, CH25Hextracellular space, cytosol

• (6R)-L-erythro-6,7-dihydrobiopterinReach 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
  3. (4aS,6R)-4a-hydroxy-L-erythro-5,6,7,8-tetrahydrobiopterin = (6R)-L-erythro-6,7-dihydrobiopterin + H2O

  Catalysed by SLC7A6, TPH1, TPH2, PCBD1 and 1 moreextracellular space, cytosol

• 2-iminopropanoateReach from here →
  1. transport of alanine and ornithine (cytosol to extracellular)
  2. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  3. 2-iminopropanoate + H2O = pyruvate + NH4(+)

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