L-citrulline

Three steps

• 3-fumarylpyruvateReach from here →
  1. L-Citrulline:L-aspartate ligase (AMP-forming)
  2. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)
  3. 3-fumarylpyruvate + H2O = fumarate + pyruvate + H(+)

  Catalysed by ASS1, ASL, FAHD1cytosolOne of 2 routes

• pantetheine 4'-phosphateReach from here →
  1. transport of citrulline and cysteine (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 citrulline (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. transport of arginine and citrulline (cytosol to extracellular)
  2. L-Arginine:glycine amidinotransferase
  3. S-Adenosyl-L-methionine:guanidinoacetate N-methyltransferase

  Catalysed by SLC7A5, SLC7A6, GATM, GAMTextracellular space, cytosolOne of 2 routes

• N-acetyl-L-aspartateReach from here →
  1. L-Citrulline:L-aspartate ligase (AMP-forming)
  2. L-citrulline + L-aspartate + ATP = 2-(N(omega)-L-arginino)succinate + AMP + diphosphate + H(+)
  3. acetyl-CoA:L-aspartate N-acetyltransferase

  Catalysed by ASS1, NAT8Lcytosol, mitochondrial matrixOne of 2 routes

• bilirubin IXalphaReach from here →
  1. transport of citrulline and cysteine (cytosol to extracellular)
  2. cystine to cysteine conversion
  3. transport of bilirubin and GSH (cytosol to extracellular)

  Catalysed by SLC7A6, SLCO1A2, SLCO1B3extracellular space, cytosolOne of 2 routes

• oxalic acidReach from here →
  1. transport of citrulline and lysine (cytosol to extracellular)
  2. transport of chloride and lysine (cytosol to extracellular)
  3. transport of chloride and oxalate (cytosol to extracellular)

  Catalysed by SLC7A5, SLC7A6, SLC6A14, SLC26A6extracellular space, cytosolOne of 3 routes

• (R)-3-hydroxybutyric acidReach from here →
  1. transport of alanine and citrulline (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 2 routes

• 2-aminoacrylic acidReach from here →
  1. transport of citrulline 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. transport of citrulline and cysteine (cytosol to extracellular)
  2. cystine to cysteine conversion
  3. transport of GSH and taurolithocholate (cytosol to extracellular)

  Catalysed by SLC7A6, SLCO1A2, SLCO1B3extracellular space, cytosolOne of 2 routes

• 3-hydroxypyruvateReach from here →
  1. transport of alanine and citrulline (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. transport of citrulline and cysteine (cytosol to extracellular)
  2. cystine to cysteine conversion
  3. glutathione:dehydroascorbate oxidoreductase

  Catalysed by SLC7A6extracellular space, cytosol

• (S)-4-hydroxymandelateReach from here →
  1. transport of citrulline 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. transport of citrulline and cysteine (cytosol to extracellular)
  2. cystine to cysteine conversion
  3. Formation of Homocystine

  Catalysed by SLC7A6extracellular space, cytosol

• anilineReach from here →
  1. transport of citrulline and lysine (cytosol to extracellular)
  2. Acetylation of Lysine
  3. Acetyl-CoA:arylamine N-acetyltransferase

  Catalysed by SLC7A5, SLC7A6, NAT2, NAT1extracellular space, cytosol, mitochondrial matrix

• aceneuramateReach from here →
  1. transport of alanine and citrulline (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. transport of citrulline and lysine (cytosol to extracellular)
  2. Acetylation of Lysine
  3. N1,N12-diacetylspermine to N1-acetylspermine conversion

  Catalysed by SLC7A5, SLC7A6extracellular space, cytosol, mitochondrial matrixOne of 2 routes

• 5'-deoxyadenosineReach from here →
  1. transport of alanine and citrulline (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 citrulline and cysteine (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-Citrulline:L-aspartate ligase (AMP-forming)
  2. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)
  3. transport of fumarate and sulfite (cytosol to mitochondria)

  Catalysed by ASS1, ASL, SLC25A10cytosol, mitochondrial matrixOne of 4 routes

• S-methyl-5'-thioadenosineReach from here →
  1. Amino Acid-Polyamine-Organocation (Apc) Tcdb:2.A.3.8.15
  2. L-ornithine carboxy-lyase (putrescine-forming)
  3. S-adenosylmethioninamine:putrescine 3-aminopropyltransferase

  Catalysed by SLC7A9, ODC1, SRMextracellular space, cytosolOne of 2 routes

• diiodineReach from here →
  1. transport of citrulline 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. transport of arginine and citrulline (cytosol to extracellular)
  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 SLC7A5, SLC7A6, NOS3, NOS1 and 2 moreextracellular space, cytosol

• 3,3',5'-triiodo-L-thyronine sulfateReach from here →
  1. transport of alanine and citrulline (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 citrulline (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 citrulline (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. transport of citrulline and lysine (cytosol to extracellular)
  2. Acetylation of Lysine
  3. Fatty Acid Beta Oxidation (C18:3->C16:3), Mitochondrial

  Catalysed by SLC7A5, SLC7A6, HADHA, ACADVL and 1 moreextracellular space, cytosol, mitochondrial matrix

• CHEBI:17724Reach from here →
  1. transport of citrulline and glycine (cytosol to extracellular)
  2. S-Adenosyl-L-methionine:glycine N-methyltransferase
  3. dimethylglycine dehydrogenase (formaldehyde)

  Catalysed by SLC7A9, SLC3A1, SLC7A6, GNMT and 2 moreextracellular space, cytosol

• glycine betaineReach from here →
  1. transport of citrulline and lysine (cytosol to extracellular)
  2. transport of chloride and lysine (cytosol to extracellular)
  3. transport of betaine and chloride (cytosol to extracellular)

  Catalysed by SLC7A5, SLC7A6, SLC6A14, SLC6A12extracellular space, cytosolOne of 3 routes

• CHEBI:17769Reach from here →
  1. Amino Acid-Polyamine-Organocation (Apc) Tcdb:2.A.3.8.15
  2. L-ornithine carboxy-lyase (putrescine-forming)
  3. Putrescine:oxygen oxidoreductase (deaminating)

  Catalysed by SLC7A9, ODC1, AOC2, AOC1 and 1 moreextracellular space, cytosolOne of 2 routes

• urateReach from here →
  1. transport of arginine and citrulline (cytosol to extracellular)
  2. L-Arginine amidinohydrolase
  3. Peroxidase

  Catalysed by SLC7A5, SLC7A6, ARG1, MPOextracellular space, cytosol, nucleusOne of 4 routes

• methylarsonous acidReach from here →
  1. transport of citrulline and glycine (cytosol to extracellular)
  2. gamma-L-glutamyl-L-cysteine + glycine + ATP = glutathione + ADP + phosphate + H(+)
  3. methylarsonate + 2 glutathione + H(+) = methylarsonous acid + glutathione disulfide + H2O

  Catalysed by SLC7A9, SLC3A1, SLC7A6, GSS and 2 moreextracellular space, cytosol

• 3-oxopropanoic acidReach from here →
  1. transport of alanine and citrulline (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

• thiocyanateReach from here →
  1. transport of citrulline and cysteine (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

• 4-fumarylacetoacetateReach from here →
  1. L-Citrulline:L-aspartate ligase (AMP-forming)
  2. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)
  3. 4-fumarylacetoacetate + H2O = acetoacetate + fumarate + H(+)

  Catalysed by ASS1, ASL, FAHcytosol

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

  Catalysed by SLC7A9, SLC3A1, SLC7A6, DDC and 3 moreextracellular space, cytosolOne of 2 routes

• CHEBI:18183Reach from here →
  1. transport of alanine and citrulline (cytosol to extracellular)
  2. gamma-glutamyltransferase (alanine)
  3. gamma-glutamylcyclotransferase ((5-L-glutamyl)-L-amino acid)

  Catalysed by SLC7A6, GGT1, GGT5, GGTLC2 and 5 moreextracellular space, cytosolOne of 2 routes

• N-carbamoyl-beta-alanineReach from here →
  1. transport of citrulline and lysine (cytosol to extracellular)
  2. transport of chloride and lysine (cytosol to extracellular)
  3. Transport of 3-Ureidopropionate

  Catalysed by SLC7A5, SLC7A6, SLC6A14extracellular space, cytosolOne of 2 routes

• N(2)-formyl-N(1)-(5-phospho-D-ribosyl)glycinamideReach from here →
  1. transport of citrulline and glycine (cytosol to extracellular)
  2. 5-Phospho-D-ribosylamine:glycine ligase (ADP-forming)
  3. 10-formyltetrahydrofolate:5'-phosphoribosylglycinamide formyltransferase

  Catalysed by SLC7A9, SLC3A1, SLC7A6, GARTextracellular space, cytosol

• arsenic triglutathioneReach from here →
  1. transport of citrulline and glycine (cytosol to extracellular)
  2. gamma-L-glutamyl-L-cysteine + glycine + ATP = glutathione + ADP + phosphate + H(+)
  3. arsenic triglutathione + 3 [thioredoxin]-dithiol + 3 S-adenosyl-L-methionine = trimethylarsine + 3 [thioredoxin]-disulfide + 3 glutathione + 3 S-adenosyl-L-homocysteine + 3 H(+)

  Catalysed by SLC7A9, SLC3A1, SLC7A6, GSS and 1 moreextracellular space, cytosolOne of 3 routes

• CHEBI:18405Reach from here →
  1. transport of alanine and citrulline (cytosol to extracellular)
  2. L-selenocysteine selenide-lyase (L-alanine-forming)
  3. pyridoxine 5'-phosphate:oxygen oxidoreductase

  Catalysed by SLC7A6, SCLY, PNPOextracellular space, cytosolOne of 3 routes

• gamma-L-glutamyl-(2S)-2-aminobutanoateReach from here →
  1. transport of alanine and citrulline (cytosol to extracellular)
  2. 2-oxobutanoate + L-alanine = (2S)-2-aminobutanoate + pyruvate
  3. (2S)-2-aminobutanoate + L-glutamate + ATP = gamma-L-glutamyl-(2S)-2-aminobutanoate + ADP + phosphate + H(+)

  Catalysed by SLC7A6, AGXT2, GCLCextracellular space, cytosol

• 2-(alpha-Hydroxypropyl)thiamine diphosphateReach from here →
  1. transport of citrulline and threonine (cytosol to extracellular)
  2. L-threonine ammonia-lyase (2-oxobutanoate-forming)
  3. branched chain keto acid dehydrogenase E1 (2-oxobutyrate)

  Catalysed by SLC7A6, SDS, SDSL, BCKDHA and 1 moreextracellular space, cytosol, mitochondrial matrix

• N-methyltryptamineReach from here →
  1. transport of citrulline and tryptophan (cytosol to extracellular)
  2. L-tryptophan decarboxy-lyase
  3. S-Adenosyl-L-methionine:amine N-methyltransferase

  Catalysed by SLC7A9, SLC3A1, SLC7A6, DDC and 1 moreextracellular space, cytosol

• D-isoleucineReach from here →
  1. transport of alanine and citrulline (cytosol to extracellular)
  2. D-serine(out) + L-alanine(in) = D-serine(in) + L-alanine(out)
  3. D-isoleucine(out) + D-serine(in) = D-isoleucine(in) + D-serine(out)

  Catalysed by SLC7A6, SLC7A10extracellular space, cytosol, inner side of the membrane, outer side of the membrane

• 15-deoxy-Delta(12,14)-prostaglandin J2-S-(R)-glutathioneReach from here →
  1. transport of citrulline and glycine (cytosol to extracellular)
  2. gamma-L-glutamyl-L-cysteine + glycine + ATP = glutathione + ADP + phosphate + H(+)
  3. 15-deoxy-Delta(12,14)-prostaglandin J2 + glutathione = 15-deoxy-Delta(12,14)-prostaglandin J2-S-(R)-glutathione

  Catalysed by SLC7A9, SLC3A1, SLC7A6, GSS and 1 moreextracellular space, cytosol

• Xaa-L-Pro dipeptideReach from here →
  1. transport of alanine and citrulline (cytosol to extracellular)
  2. L-proline(out) + L-alanine(in) + Na(+)(out) = L-proline(in) + L-alanine(out) + Na(+)(in)
  3. Xaa-L-Pro dipeptide + H2O = an L-alpha-amino acid + L-proline

  Catalysed by SLC7A6, SLC1A4, PEPDextracellular space, cytosol, inner side of the membrane, outer side of the membrane

• (6R)-10-formyltetrahydrofolateReach from here →
  1. transport of citrulline and glycine (cytosol to extracellular)
  2. (6R)-5,10-methylene-5,6,7,8-tetrahydrofolate + glycine + H2O = (6S)-5,6,7,8-tetrahydrofolate + L-serine
  3. (6S)-5,6,7,8-tetrahydrofolate + formate + ATP = (6R)-10-formyltetrahydrofolate + ADP + phosphate

  Catalysed by SLC7A9, SLC3A1, SLC7A6, SHMT1 and 3 moreextracellular space, cytosol

• (5S)-hydroxy-(6E,8Z,11Z,14Z,17Z)-eicosapentaenoateReach from here →
  1. transport of citrulline and glycine (cytosol to extracellular)
  2. gamma-L-glutamyl-L-cysteine + glycine + ATP = glutathione + ADP + phosphate + H(+)
  3. (5S)-hydroperoxy-(6E,8Z,11Z,14Z,17Z)-eicosapentaenoate + 2 glutathione = (5S)-hydroxy-(6E,8Z,11Z,14Z,17Z)-eicosapentaenoate + glutathione disulfide + H2O

  Catalysed by SLC7A9, SLC3A1, SLC7A6, GSS and 2 moreextracellular space, cytosol