L-citrulline

Three steps

• all-trans-retinoic acidReach from here →
  1. transport of alanine and citrulline (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 2 routes

• CHEBI:15380Reach from here →
  1. transport of citrulline and lysine (cytosol to extracellular)
  2. Acetylation of Lysine
  3. 5-aminolevulinate synthase (2-amino-3-oxoadipate)

  Catalysed by SLC7A5, SLC7A6, ALAS1, CPNE7extracellular space, cytosol, mitochondrial matrixOne of 8 routes

• CHEBI:15477Reach from here →
  1. transport of citrulline and lysine (cytosol to extracellular)
  2. Acetylation of Lysine
  3. glycine N-acyltransferase (2-methylbutyryl-CoA)

  Catalysed by SLC7A5, SLC7A6, GLYAT, GLYATL2 and 1 moreextracellular space, cytosol, mitochondrial matrixOne of 3 routes

• 2-methylcrotonoyl-CoAReach from here →
  1. transport of citrulline and lysine (cytosol to extracellular)
  2. Acetylation of Lysine
  3. Production of Tiglylcarnitine

  Catalysed by SLC7A5, SLC7A6, CPT1A, CPT1Bextracellular space, cytosol, mitochondrial matrixOne of 2 routes

• isobutyryl-CoAReach from here →
  1. transport of citrulline and lysine (cytosol to extracellular)
  2. Acetylation of Lysine
  3. acetyl-CoA C-acyltransferase (3-oxo-4-methyl-pentanoyl-CoA)

  Catalysed by SLC7A5, SLC7A6, HADHA, ACAA2 and 1 moreextracellular space, cytosol, mitochondrial matrixOne of 3 routes

• 3-methylbut-2-enoyl-CoAReach from here →
  1. transport of citrulline and lysine (cytosol to extracellular)
  2. Acetylation of Lysine
  3. glycine-N-acyltransferase (3-methylcrotonoylglycine)

  Catalysed by SLC7A5, SLC7A6, GLYATextracellular space, cytosol, mitochondrial matrix

• isovaleryl-CoAReach from here →
  1. transport of citrulline and lysine (cytosol to extracellular)
  2. Acetylation of Lysine
  3. glycine N-acyltransferase (isovalerylglycine)

  Catalysed by SLC7A5, SLC7A6, GLYAT, GLYATL2 and 1 moreextracellular space, cytosol, mitochondrial matrixOne of 3 routes

• arachidonoyl-CoAReach from here →
  1. transport of citrulline and lysine (cytosol to extracellular)
  2. Acetylation of Lysine
  3. Arachidonate:CoA ligase (AMP-forming)

  Catalysed by SLC7A5, SLC7A6, SLC27A2, ACSL4 and 6 moreextracellular space, cytosol, mitochondrial matrixOne of 11 routes

• CHEBI:15519Reach from here →
  1. transport of citrulline and lysine (cytosol to extracellular)
  2. Acetylation of Lysine
  3. propanoyl-CoA C-acyltransferase

  Catalysed by SLC7A5, SLC7A6, ACAA1, SCP2 and 1 moreextracellular space, cytosol, mitochondrial matrix, peroxisomeOne of 2 routes

• palmitoyl-CoAReach from here →
  1. transport of citrulline and lysine (cytosol to extracellular)
  2. Acetylation of Lysine
  3. Palmitate:CoA ligase (AMP-forming)

  Catalysed by SLC7A5, SLC7A6, SLC27A2, ACSL4 and 6 moreextracellular space, cytosol, mitochondrial matrixOne of 11 routes

• oleoyl-CoAReach from here →
  1. transport of citrulline and lysine (cytosol to extracellular)
  2. Acetylation of Lysine
  3. Transport of octadecenoyl-CoA into Mitochondrial Matrix

  Catalysed by SLC7A5, SLC7A6, CPT1A, CPT1C and 1 moreextracellular space, cytosol, mitochondrial matrixOne of 11 routes

• phenylacetyl-CoAReach from here →
  1. transport of citrulline and lysine (cytosol to extracellular)
  2. Acetylation of Lysine
  3. Phenylacetate:CoA ligase

  Catalysed by SLC7A5, SLC7A6extracellular space, cytosol, mitochondrial matrixOne of 4 routes

• propionyl-CoAReach from here →
  1. transport of citrulline and lysine (cytosol to extracellular)
  2. Acetylation of Lysine
  3. Propanoate:CoA ligase (AMP-forming)

  Catalysed by SLC7A5, SLC7A6, ACSS3, ACSS2 and 1 moreextracellular space, cytosol, mitochondrial matrixOne of 44 routes

• (S)-malateReach from here →
  1. L-Citrulline:L-aspartate ligase (AMP-forming)
  2. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)
  3. (S)-malate hydro-lyase (fumarate-forming)

  Catalysed by ASS1, ASL, FHcytosolOne of 9 routes

• (6R)-5,10-methylene-5,6,7,8-tetrahydrofolateReach 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. (6R)-5,10-methylene-5,6,7,8-tetrahydrofolate + glycine + H2O = (6S)-5,6,7,8-tetrahydrofolate + L-serine

  Catalysed by SLC7A9, SLC3A1, SLC7A6, SHMT1 and 1 moreextracellular space, cytosolOne of 2 routes

• 10-formyltetrahydrofolic acidReach from here →
  1. transport of citrulline and glycine (cytosol to extracellular)
  2. 5,10-Methylenetetrahydrofolate:glycine hydroxymethyltransferase
  3. Formate:tetrahydrofolate ligase (ADP-forming)

  Catalysed by SLC7A9, SLC3A1, SLC7A6, SHMT1 and 1 moreextracellular space, cytosolOne of 3 routes

• 5-methyltetrahydrofolic acidReach from here →
  1. transport of citrulline and glycine (cytosol to extracellular)
  2. 5,10-Methylenetetrahydrofolate:glycine hydroxymethyltransferase
  3. histamine N-methyltransferase (SAM)

  Catalysed by SLC7A9, SLC3A1, SLC7A6, SHMT1 and 1 moreextracellular space, cytosol

• CHEBI:15650Reach from here →
  1. transport of citrulline and lysine (cytosol to extracellular)
  2. Acetylation of Lysine
  3. N-acetyl-LTE4 to leukotriene E4 conversion

  Catalysed by SLC7A5, SLC7A6, ZDHHC11B, NAA10 and 30 moreextracellular space, cytosol, mitochondrial matrixOne of 6 routes

• betaine aldehydeReach 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. choline + A = betaine aldehyde + AH2

  Catalysed by SLC7A6, MOCOS, CHDHextracellular space, cytosol

• formateReach from here →
  1. transport of citrulline 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 SLC7A9, SLC3A1, SLC7A6, IDO1 and 3 moreextracellular space, cytosol

• CHEBI:15746Reach from here →
  1. transport of citrulline and lysine (cytosol to extracellular)
  2. Acetylation of Lysine
  3. diamine N-acetyltransferase (N1-acetylspermine)

  Catalysed by SLC7A5, SLC7A6, SAT1, SAT2extracellular space, cytosol, mitochondrial matrixOne of 2 routes

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

  Catalysed by SLC7A9, SLC3A1, SLC7A6, SLC7A5 and 3 moreextracellular space, cytosol

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

  Catalysed by SLC7A9, SLC3A1, SLC7A6, SLC7A5 and 1 moreextracellular space, cytosol

• bromideReach from here →
  1. transport of citrulline and cysteine (cytosol to extracellular)
  2. cystine to cysteine conversion
  3. 2-bromoacetaldehyde:glutathione S-(formylmethyl)transferase

  Catalysed by SLC7A6, MGST3, GSTA4, GSTA1 and 14 moreextracellular space, cytosolOne of 2 routes

• keto-D-fructose 6-phosphateReach from here →
  1. Carbamoyl-phosphate:L-ornithine carbamoyltransferase
  2. glucose-6-phosphatase (carbamoyl-phosphate)
  3. D-glucose-6-phosphate aldose-ketose-isomerase

  Catalysed by OTC, G6PC2, GPImitochondrial matrix, endoplasmic reticulum, cytosolOne of 2 routes

• thioredoxin dithiolReach from here →
  1. transport of alanine and citrulline (cytosol to extracellular)
  2. L-Alanine:tRNA(Ala) ligase (AMP-forming)
  3. glycyl-tRNA(gly) phosphorylation

  Catalysed by SLC7A6, AARS1extracellular space, cytosolOne of 18 routes

• methanethiolReach from here →
  1. transport of citrulline and cysteine (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. transport of citrulline and glycine (cytosol to extracellular)
  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 SLC7A9, SLC3A1, SLC7A6, GSS and 23 moreextracellular space, cytosol

• CHEBI:16132Reach from here →
  1. transport of citrulline 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 citrulline 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-Citrulline:L-aspartate ligase (AMP-forming)
  2. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)
  3. transport of fumarate and sulfate (cytosol to mitochondria)

  Catalysed by ASS1, ASL, SLC25A10cytosol, mitochondrial matrixOne of 6 routes

• 2-methyl-3-oxopropanoic acidReach from here →
  1. transport of alanine and citrulline (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. transport of arginine and citrulline (cytosol to extracellular)
  2. L-Arginine, NADPH:Oxygen Oxidoreductase (Nitric-Oxide-Forming)
  3. 4 nitric oxide + O2 + 2 H2O = 4 nitrite + 4 H(+)

  Catalysed by SLC7A5, SLC7A6, NOS3, NOS1 and 2 moreextracellular space, cytosol

• 11-cis-retinolReach from here →
  1. transport of citrulline and lysine (cytosol to extracellular)
  2. Acetylation of Lysine
  3. Palmitoyl-CoA:retinol O-acyltransferase

  Catalysed by SLC7A5, SLC7A6, DGAT1, LRAT and 3 moreextracellular space, cytosol, mitochondrial matrix

• cob(II)alaminReach 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. 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. transport of citrulline and cysteine (cytosol to extracellular)
  2. cystine to cysteine conversion
  3. transport of GSH and lithocholate (cytosol to extracellular)

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

• ubiquinonesReach from here →
  1. L-Citrulline:L-aspartate ligase (AMP-forming)
  2. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)
  3. succinate:quinone oxidoreductase

  Catalysed by ASS1, ASL, SDHD, SDHB and 2 morecytosol, mitochondrial matrix

• 2-phenylacetaldehydeReach from here →
  1. transport of citrulline 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

• oxaloacetateReach 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. L-Aspartate:2-oxoglutarate aminotransferase

  Catalysed by ASS1, GOT2cytosol, mitochondrial matrixOne of 10 routes

• selaneReach from here →
  1. transport of alanine and citrulline (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 citrulline (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. transport of alanine and citrulline (cytosol to extracellular)
  2. alanine racemase
  3. Formation of Alanylalanine

  Catalysed by SLC7A6extracellular space, cytosol

• (S)-lactateReach from here →
  1. transport of alanine and citrulline (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 citrulline and cysteine (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 citrulline (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. transport of arginine and citrulline (cytosol to extracellular)
  2. L-Arginine amidinohydrolase
  3. urea = cyanamide + H2O

  Catalysed by SLC7A5, SLC7A6, ARG1, CA1 and 1 moreextracellular space, cytosol

• pyridoxineReach from here →
  1. transport of alanine and citrulline (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 citrulline (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 citrulline 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. transport of citrulline and cysteine (cytosol to extracellular)
  2. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  3. N(6),N(6)-dimethyl-L-lysyl(4)-[histone H3] + 2 A + 2 H2O = L-lysyl(4)-[histone H3] + 2 formaldehyde + 2 AH2

  Catalysed by SLC7A6, MOCOS, KDM1A, KDM1Bextracellular space, cytosolOne of 5 routes