L-citrulline

Three steps

• 4,8,12-trimethyltridecanoyl-CoAReach from here →
  1. transport of citrulline and lysine (cytosol to extracellular)
  2. Acetylation of Lysine
  3. propanoyl-CoA C-acyltransferase (3-ketopristanoyl-CoA)

  Catalysed by SLC7A5, SLC7A6, ACAA1, MYO5Bextracellular space, cytosol, mitochondrial matrix, peroxisomeOne of 2 routes

• (6Z,9Z,12Z)-octadecatrienoyl-CoAReach from here →
  1. transport of citrulline and lysine (cytosol to extracellular)
  2. Acetylation of Lysine
  3. long-chain-fatty-acid-CoA ligase (gamma-linolenate)

  Catalysed by SLC7A5, SLC7A6, SLC27A2, ACSL4 and 6 moreextracellular space, cytosol, mitochondrial matrixOne of 7 routes

• benzoyl-CoAReach from here →
  1. transport of citrulline and glycine (cytosol to extracellular)
  2. Hippurate (Benzoylglycinate) Formation
  3. benzoyl-CoA + glycine = N-benzoylglycine + CoA + H(+)

  Catalysed by SLC7A9, SLC3A1, SLC7A6, GLYATextracellular space, cytosol, mitochondrial matrix

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

  Catalysed by SLC7A5, SLC7A6, ACSM4, ACSM1 and 4 moreextracellular space, cytosol, mitochondrial matrixOne of 9 routes

• dodecanoyl-CoAReach from here →
  1. transport of citrulline and lysine (cytosol to extracellular)
  2. Acetylation of Lysine
  3. long-chain-fatty-acid-CoA ligase (lauric acid)

  Catalysed by SLC7A5, SLC7A6, SLC27A2, ACSL4 and 6 moreextracellular space, cytosol, mitochondrial matrixOne of 10 routes

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

  Catalysed by SLC7A5, SLC7A6, CPT1A, CPT1C and 1 moreextracellular space, cytosol, mitochondrial matrixOne of 2 routes

• hexadecanoyl-CoAReach from here →
  1. transport of citrulline and glycine (cytosol to extracellular)
  2. hexadecanoyl-CoA + glycine = N-hexadecanoylglycine + CoA + H(+)
  3. hexadecanoyl-CoA + glycine = N-hexadecanoylglycine + CoA + H(+)

  Catalysed by SLC7A9, SLC3A1, SLC7A6, GLYATL3extracellular space, cytosol

• eicosanoyl-CoAReach from here →
  1. transport of citrulline and lysine (cytosol to extracellular)
  2. Acetylation of Lysine
  3. long-chain-fatty-acid-CoA ligase (eicosanoate)

  Catalysed by SLC7A5, SLC7A6, SLC27A2, ACSL4 and 6 moreextracellular space, cytosol, mitochondrial matrixOne of 12 routes

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

  Catalysed by SLC7A5, SLC7A6, SUCLG1, SUCLG2 and 1 moreextracellular space, cytosol, mitochondrial matrixOne of 2 routes

• (9Z,12Z)-octadecadienoyl-CoAReach from here →
  1. transport of citrulline and lysine (cytosol to extracellular)
  2. Acetylation of Lysine
  3. long-chain-fatty-acid-CoA ligase ((6Z,9Z)-octadecadienoic acid)

  Catalysed by SLC7A5, SLC7A6, SLC27A2, ACSL4 and 6 moreextracellular space, cytosol, mitochondrial matrixOne of 14 routes

• tetradecanoyl-CoAReach from here →
  1. transport of citrulline and lysine (cytosol to extracellular)
  2. Acetylation of Lysine
  3. long-chain-fatty-acid-CoA ligase (myristic acid)

  Catalysed by SLC7A5, SLC7A6, SLC27A2, ACSL4 and 6 moreextracellular space, cytosol, mitochondrial matrixOne of 12 routes

• octanoyl-CoAReach from here →
  1. transport of citrulline and lysine (cytosol to extracellular)
  2. Acetylation of Lysine
  3. Octanoyl-CoA:L-carnitine O-octanoyltransferase

  Catalysed by SLC7A5, SLC7A6, CROTextracellular space, cytosol, mitochondrial matrixOne of 21 routes

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

  Catalysed by SLC7A5, SLC7A6extracellular space, cytosol, mitochondrial matrixOne of 5 routes

• octadecanoyl-CoAReach from here →
  1. transport of citrulline and lysine (cytosol to extracellular)
  2. Acetylation of Lysine
  3. long-chain-fatty-acid-CoA ligase (stearate)

  Catalysed by SLC7A5, SLC7A6, SLC27A2, ACSL4 and 5 moreextracellular space, cytosol, mitochondrial matrixOne of 15 routes

• prostaglandin E1Reach from here →
  1. transport of citrulline and cysteine (cytosol to extracellular)
  2. cystine to cysteine conversion
  3. transport of GSH and prostaglandin E1 (cytosol to extracellular)

  Catalysed by SLC7A6, SLCO2A1extracellular space, cytosolOne of 2 routes

• prostaglandin I2Reach from here →
  1. transport of citrulline and cysteine (cytosol to extracellular)
  2. cystine to cysteine conversion
  3. transport of GSH and prostaglandin I2 (cytosol to extracellular)

  Catalysed by SLC7A6, SLCO2A1extracellular space, cytosolOne of 2 routes

• prostaglandin F2alphaReach from here →
  1. transport of citrulline and cysteine (cytosol to extracellular)
  2. cystine to cysteine conversion
  3. transport of GSH and prostaglandin F2alpha (cytosol to extracellular)

  Catalysed by SLC7A6, SLCO2A1extracellular space, cytosolOne of 2 routes

• prostaglandin H2Reach 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. (5Z,8Z,11Z,14Z)-eicosatetraenoate + AH2 + 2 O2 = prostaglandin H2 + A + H2O

  Catalysed by SLC7A6, MOCOS, PTGS1, PTGS2extracellular space, cytosolOne of 4 routes

• prostaglandin D2Reach from here →
  1. transport of citrulline and cysteine (cytosol to extracellular)
  2. cystine to cysteine conversion
  3. transport of GSH and prostaglandin D2 (cytosol to extracellular)

  Catalysed by SLC7A6, SLCO2A1extracellular space, cytosolOne of 2 routes

• (15S)-hydroxy-(5Z,8Z,11Z,13E)-eicosatetraenoateReach 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. (15S)-hydroperoxy-(5Z,8Z,11Z,13E)-eicosatetraenoate + AH2 = (15S)-hydroxy-(5Z,8Z,11Z,13E)-eicosatetraenoate + A + H2O

  Catalysed by SLC7A6, MOCOS, TBXAS1, PTGS2 and 1 moreextracellular space, cytosolOne of 3 routes

• 7,8-dihydrofolateReach 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. uridine(34) in tRNA + 5,10-methylenetetrahydrofolate + taurine + GTP + A + H2O = 5-taurinomethyluridine(34) in tRNA + 7,8-dihydrofolate + GDP + AH2 + phosphate + H(+)

  Catalysed by SLC7A6, MOCOS, MTO1extracellular space, cytosolOne of 4 routes

• 5-formimidoyltetrahydrofolateReach from here →
  1. transport of citrulline and glycine (cytosol to extracellular)
  2. 5,10-Methylenetetrahydrofolate:glycine hydroxymethyltransferase
  3. 5-Formiminotetrahydrofolate:L-glutamate N-formiminotransferase

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

• leukotriene A4Reach from here →
  1. transport of citrulline and cysteine (cytosol to extracellular)
  2. cystine to cysteine conversion
  3. transport of GSH and leukotriene A4 (cytosol to extracellular)

  Catalysed by SLC7A6, SLCO2A1extracellular space, cytosolOne of 3 routes

• (R)-S-lactoylglutathioneReach from here →
  1. transport of citrulline and cysteine (cytosol to extracellular)
  2. cystine to cysteine conversion
  3. (R)-S-Lactoylglutathione methylglyoxal-lyase (isomerizing)

  Catalysed by SLC7A6, GLO1, GLOD4extracellular space, cytosolOne of 3 routes

• carnosineReach from here →
  1. Amino Acid-Polyamine-Organocation (Apc) Tcdb:2.A.3.8.15
  2. L-histidine(in) + L-ornithine(out) + H(+)(in) = L-histidine(out) + L-ornithine(in) + H(+)(out)
  3. beta-alanine + L-histidine + ATP = carnosine + ADP + phosphate + H(+)

  Catalysed by SLC7A9, SLC25A2, CARNS1extracellular space, cytosol, inner side of the membrane, outer side of the membraneOne of 2 routes

• 2-oxoadipateReach from here →
  1. transport of citrulline and glycine (cytosol to extracellular)
  2. glycine + 2-oxoglutarate = glyoxylate + L-glutamate
  3. L-2-aminoadipate + glyoxylate = 2-oxoadipate + glycine

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

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

  Catalysed by SLC7A6, TPOextracellular space, cytosolOne of 2 routes

• N-acetyl-D-glucosamine 6-phosphateReach from here →
  1. transport of citrulline and glutamine (cytosol to extracellular)
  2. L-glutamine:D-fructose-6-phosphate isomerase (deaminating)
  3. acetyl-CoA:D-glucosamine-6-phosphate N-acetyltransferase

  Catalysed by SLC7A6, GFPT2, GFPT1, GNPNAT1extracellular space, cytosolOne of 3 routes

• N(6)-(1,2-dicarboxyethyl)-AMPReach from here →
  1. L-Citrulline:L-aspartate ligase (AMP-forming)
  2. 2-(Nomega-L-arginino)succinate arginine-lyase (fumarate-forming)
  3. N6-(1,2-dicarboxyethyl)AMP AMP-lyase (fumarate-forming)

  Catalysed by ASS1, ASL, ADSL, TNRC6BcytosolOne of 4 routes

• beta-D-fructose 6-phosphateReach from here →
  1. transport of citrulline and glutamine (cytosol to extracellular)
  2. L-glutamine:D-fructose-6-phosphate isomerase (deaminating)
  3. alpha-D-glucosamine 6-phosphate + H2O = beta-D-fructose 6-phosphate + NH4(+)

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

• S-formylglutathioneReach from here →
  1. transport of citrulline and cysteine (cytosol to extracellular)
  2. cystine to cysteine conversion
  3. alcohol dehydrogenase (formaldehyde)

  Catalysed by SLC7A6, ADH1C, ADH1A, ADH5 and 3 moreextracellular space, cytosolOne of 2 routes

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

  Catalysed by SLC7A6, AGXT2extracellular space, cytosol, mitochondrial matrix

• sphing-4-enineReach from here →
  1. transport of citrulline and lysine (cytosol to extracellular)
  2. Acetylation of Lysine
  3. acyl-CoA:sphingosine N-acyltransferase

  Catalysed by SLC7A5, SLC7A6, ASAH1, ACER2 and 2 moreextracellular space, cytosol, mitochondrial matrix, Golgi apparatusOne of 2 routes

• spermidineReach 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 8 routes

• hypotaurineReach from here →
  1. transport of citrulline and cysteine (cytosol to extracellular)
  2. L-Cysteine:oxygen oxidoreductase
  3. 3-sulfino-L-alanine + H(+) = hypotaurine + CO2

  Catalysed by SLC7A6, CDO1, GADL1, CSADextracellular space, cytosol

• 6-phospho-D-glucono-1,5-lactoneReach from here →
  1. Carbamoyl-phosphate:L-ornithine carbamoyltransferase
  2. glucose-6-phosphatase (carbamoyl-phosphate)
  3. D-glucose-6-phosphate:NADP+ 1-oxidoreductase

  Catalysed by OTC, G6PC2, G6PDmitochondrial matrix, endoplasmic reticulumOne of 3 routes

• dimethylamineReach from here →
  1. Amino Acid-Polyamine-Organocation (Apc) Tcdb:2.A.3.8.15
  2. N(omega),N(omega)-dimethyl-L-arginine(in) + L-ornithine(out) = N(omega),N(omega)-dimethyl-L-arginine(out) + L-ornithine(in)
  3. Nomega,Nomega'-dimethyl-L-arginine dimethylamidohydrolase

  Catalysed by SLC7A9, SLC25A2, DDAH1extracellular space, cytosol, inner side of the membrane, outer side of the membraneOne of 2 routes

• N(4)-(beta-N-acetyl-D-glucosaminyl)-L-asparagineReach 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. N(4)-(beta-N-acetyl-D-glucosaminyl)-L-asparagine + H2O = N-acetyl-beta-D-glucosaminylamine + L-aspartate + H(+)

  Catalysed by ASS1, AGAcytosol

• porphobilinogenReach from here →
  1. transport of citrulline and glycine (cytosol to extracellular)
  2. succinyl-CoA:glycine C-succinyltransferase (decarboxylating)
  3. 5-aminolevulinate hydro-lyase (adding 5-aminolevulinate and cyclizing

  Catalysed by SLC7A9, SLC3A1, SLC7A6, ALAS1 and 3 moreextracellular space, cytosol, mitochondrial matrixOne of 2 routes

• L,L-cystathionineReach from here →
  1. transport of citrulline and lysine (cytosol to extracellular)
  2. Acetylation of Lysine
  3. N-acetylglutamate synthase (N-acetyl-L-cystathionine)

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

• phosphoethanolamineReach from here →
  1. transport of citrulline and threonine (cytosol to extracellular)
  2. L-threonine acetaldehyde-lyase (glycine-forming)
  3. phosphoethanolamine + H2O = acetaldehyde + NH4(+) + phosphate

  Catalysed by SLC7A6, ETNPPLextracellular space, cytosol

• N,N-dimethylglycineReach from here →
  1. transport of citrulline and methionine (cytosol to extracellular)
  2. S-methyl-L-methionine + L-homocysteine = 2 L-methionine + H(+)
  3. L-homocysteine + glycine betaine = N,N-dimethylglycine + L-methionine

  Catalysed by SLC7A6, BHMT2, BHMTextracellular space, cytosolOne of 2 routes

• N-acetylputrescineReach from here →
  1. Amino Acid-Polyamine-Organocation (Apc) Tcdb:2.A.3.8.15
  2. L-ornithine carboxy-lyase (putrescine-forming)
  3. acetyl-CoA:putrescine N-acetyltransferase

  Catalysed by SLC7A9, ODC1, SAT1, SAT2extracellular space, cytosolOne of 2 routes

• 4-aminobutanalReach from here →
  1. Amino Acid-Polyamine-Organocation (Apc) Tcdb:2.A.3.8.15
  2. L-ornithine carboxy-lyase (putrescine-forming)
  3. putrescine + O2 + H2O = 4-aminobutanal + H2O2 + NH4(+)

  Catalysed by SLC7A9, ODC1, AOC1extracellular space, cytosol

• L-glutamyl 5-phosphateReach from here →
  1. Amino Acid-Polyamine-Organocation (Apc) Tcdb:2.A.3.8.15
  2. L-ornithine + 2-oxoglutarate = L-glutamate 5-semialdehyde + L-glutamate
  3. L-glutamate 5-semialdehyde + phosphate + NADP(+) = L-glutamyl 5-phosphate + NADPH + H(+)

  Catalysed by SLC7A9, OAT, ALDH18A1extracellular space, cytosol

• glutathione disulfideReach from here →
  1. transport of arginine and citrulline (cytosol to extracellular)
  2. L-cystine(out) + L-arginine(in) = L-cystine(in) + L-arginine(out)
  3. cystine to cysteine conversion

  Catalysed by SLC7A5, SLC7A6, SLC7A9extracellular space, cytosol, inner side of the membrane, outer side of the membraneOne of 36 routes

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

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

• (S)-2-amino-6-oxohexanoateReach from here →
  1. transport of citrulline and lysine (cytosol to extracellular)
  2. N6-(L-1,3-Dicarboxypropyl)-L-lysine:NADP+ oxidoreductase (L-lysine-forming)
  3. L-saccharopine + NAD(+) + H2O = (S)-2-amino-6-oxohexanoate + L-glutamate + NADH + H(+)

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

• N(1)-acetylspermidineReach from here →
  1. Amino Acid-Polyamine-Organocation (Apc) Tcdb:2.A.3.8.15
  2. L-ornithine carboxy-lyase (putrescine-forming)
  3. N(1)-acetylspermidine + O2 + H2O = 3-acetamidopropanal + putrescine + H2O2

  Catalysed by SLC7A9, ODC1, PAOXextracellular space, cytosol

• an acyl-CoAReach from here →
  1. transport of citrulline and lysine (cytosol to extracellular)
  2. Acetylation of Lysine
  3. acyl-CoA:sphingosine N-acyltransferase

  Catalysed by SLC7A5, SLC7A6, ASAH1, ACER2 and 2 moreextracellular space, cytosol, mitochondrial matrix, Golgi apparatusOne of 3 routes