L-citrulline

Three steps

• Asp-tRNA(Asp)Reach 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:tRNA(Asp) ligase (AMP-forming)

  Catalysed by ASS1, DARS1cytosol

• hypoioditeReach from here →
  1. transport of citrulline 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 citrulline and cysteine (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 citrulline (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. transport of citrulline and cysteine (cytosol to extracellular)
  2. cystine to cysteine conversion
  3. solute carrier organic anion transporter (cholate)

  Catalysed by SLC7A6, SLCO1A2, SLCO1B3extracellular space, cytosolOne of 3 routes

• glycolateReach from here →
  1. transport of citrulline and glycine (cytosol to extracellular)
  2. glycine:oxygen oxidoreductase (deaminating)
  3. glycolate:NADP+ oxidoreductase

  Catalysed by SLC7A9, SLC3A1, SLC7A6, DAO and 1 moreextracellular space, cytosol, peroxisomeOne of 3 routes

• D-aspartateReach from here →
  1. transport of citrulline and cysteine (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

• acetateReach from here →
  1. Amino Acid-Polyamine-Organocation (Apc) Tcdb:2.A.3.8.15
  2. N2-Acetyl-L-ornithine amidohydrolase
  3. N2-Acetyl-L-ornithine amidohydrolase

  Catalysed by SLC7A9, ACY1extracellular space, cytosolOne of 7 routes

• 3-acetamidopropanalReach from here →
  1. transport of arginine and citrulline (cytosol to extracellular)
  2. Transport by Y+Lat-2 into Extracellular Space
  3. N1-acetylspermidine:oxygen oxidoreductase (3-acetamidopropanal-forming)

  Catalysed by SLC7A5, SLC7A6, SLC3A2, PAOX and 1 moreextracellular space, cytosolOne of 2 routes

• oxalateReach from here →
  1. transport of citrulline and glycine (cytosol to extracellular)
  2. glycine + 2-oxoglutarate = glyoxylate + L-glutamate
  3. glyoxylate + O2 + H2O = oxalate + H2O2 + H(+)

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

• formic 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 formate (cytosol to extracellular)

  Catalysed by SLC7A5, SLC7A6, SLC6A14, SLC26A4extracellular space, cytosolOne of 5 routes

• anthranilic acidReach from here →
  1. transport of alanine and citrulline (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 citrulline (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 2 routes

• butyric acidReach from here →
  1. transport of alanine and citrulline (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 2 routes

• malonic acidReach from here →
  1. transport of citrulline and lysine (cytosol to extracellular)
  2. transport of chloride and lysine (cytosol to extracellular)
  3. Malonate Transport, Antiport with Chloride

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

• (9Z)-octadecenoateReach from here →
  1. transport of citrulline and phenylalanine (cytosol to extracellular)
  2. L-phenylalanine + (9Z)-octadecenoate = N-(9Z-octadecenoyl)-L-phenylalanine + H2O
  3. L-phenylalanine + (9Z)-octadecenoate = N-(9Z-octadecenoyl)-L-phenylalanine + H2O

  Catalysed by SLC7A6, PM20D1extracellular space, cytosolOne of 3 routes

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

• (S)-dihydroorotateReach from here →
  1. Carbamoyl-phosphate:L-ornithine carbamoyltransferase
  2. carbamoyl-phosphate:L-aspartate carbamoyltransferase
  3. (S)-dihydroorotate amidohydrolase

  Catalysed by OTC, CADmitochondrial matrix, cytosolOne of 2 routes

• an alcoholReach from here →
  1. transport of citrulline and glycine (cytosol to extracellular)
  2. gamma-L-glutamyl-L-cysteine + glycine + ATP = glutathione + ADP + phosphate + H(+)
  3. a hydroperoxide + 2 glutathione = an alcohol + glutathione disulfide + H2O

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

• glutarateReach from here →
  1. transport of citrulline and glycine (cytosol to extracellular)
  2. bile acid-CoA: amino acid N-acyltransferase
  3. glycocholate(out) + glutarate(in) = glycocholate(in) + glutarate(out)

  Catalysed by SLC7A9, SLC3A1, SLC7A6, BAAT and 1 moreextracellular space, cytosol, peroxisome, inner side of the membrane, outer side of the membraneOne of 2 routes

• (5Z,8Z,11Z,14Z)-eicosatetraenoateReach from here →
  1. transport of citrulline and phenylalanine (cytosol to extracellular)
  2. (5Z,8Z,11Z,14Z)-eicosatetraenoate + L-phenylalanine = N-(5Z,8Z,11Z,14Z-eicosatetraenoyl)-L-phenylalanine + H2O
  3. (5Z,8Z,11Z,14Z)-eicosatetraenoate + L-phenylalanine = N-(5Z,8Z,11Z,14Z-eicosatetraenoyl)-L-phenylalanine + H2O

  Catalysed by SLC7A6, PM20D1extracellular space, cytosolOne of 4 routes

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

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

• tetra-mu3-sulfido-tetrairon(2+)Reach from here →
  1. transport of citrulline and cysteine (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. transport of citrulline and cysteine (cytosol to extracellular)
  2. cystine to cysteine conversion
  3. transport of GSH and serotonin (cytosol to extracellular)

  Catalysed by SLC7A6, SLCO1A2, SLCO1B3extracellular space, cytosolOne of 5 routes

• L-alpha-aminobutyric acidReach from here →
  1. transport of citrulline 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. transport of citrulline and cysteine (cytosol to extracellular)
  2. cystine to cysteine conversion
  3. solute carrier organic anion transporter (GSH)

  Catalysed by SLC7A6, SLCO1A2, SLCO1B3extracellular space, cytosolOne of 3 routes

• 3-hydroxyanthranilateReach from here →
  1. transport of alanine and citrulline (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 citrulline and tryptophan (cytosol to extracellular)
  2. L-tryptophan:oxygen 2,3-oxidoreductase (decyclizing)
  3. Formylkynurenine hydrolase

  Catalysed by SLC7A9, SLC3A1, SLC7A6, IDO1 and 3 moreextracellular space, cytosol

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

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

• D-glucoseReach from here →
  1. Carbamoyl-phosphate:L-ornithine carbamoyltransferase
  2. glucose-6-phosphatase (carbamoyl-phosphate)
  3. D-Glucose-6-phosphate phosphohydrolase

  Catalysed by OTC, G6PC2, G6PC1, G6PC3mitochondrial matrix, endoplasmic reticulum

• (R)-lactic acidReach from here →
  1. transport of alanine and citrulline (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 citrulline (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 5 routes

• 25-hydroxycholesterolReach 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. 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 citrulline and phenylalanine (cytosol to extracellular)
  2. (6R)-L-erythro-5,6,7,8-tetrahydrobiopterin + L-phenylalanine + O2 = (4aS,6R)-4a-hydroxy-L-erythro-5,6,7,8-tetrahydrobiopterin + L-tyrosine
  3. (4aS,6R)-4a-hydroxy-L-erythro-5,6,7,8-tetrahydrobiopterin = (6R)-L-erythro-6,7-dihydrobiopterin + H2O

  Catalysed by SLC7A6, PAH, PCBD1, PCBD2extracellular space, cytosol

• 2-iminopropanoateReach from here →
  1. transport of alanine and citrulline (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

• phosphoenolpyruvic acidReach 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

• N-acetyl-beta-neuraminic acidReach from here →
  1. transport of alanine and citrulline (cytosol to extracellular)
  2. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  3. N-Acetylneuraminate pyruvate-lyase (N-acetyl-D-mannosamine-forming)

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

• tert-butanolReach from here →
  1. transport of citrulline and glycine (cytosol to extracellular)
  2. gamma-L-glutamyl-L-cysteine + glycine + ATP = glutathione + ADP + phosphate + H(+)
  3. tert-butyl hydroperoxide + 2 glutathione = tert-butanol + glutathione disulfide + H2O

  Catalysed by SLC7A9, SLC3A1, SLC7A6, GSS and 5 moreextracellular space, cytosol

• 2-methyl-1-hydroxypropylthiamine diphosphateReach from here →
  1. transport of citrulline and valine (cytosol to extracellular)
  2. L-Valine:2-oxoglutarate aminotransferase
  3. branched chain keto acid dehydrogenase E1 (3-methyl-2-oxobutyrate)

  Catalysed by SLC7A9, SLC3A1, SLC7A6, BCAT2 and 2 moreextracellular space, cytosol, mitochondrial matrix

• 1,2-dihydroxy-5-(methylsulfanyl)pent-1-en-3-oneReach from here →
  1. transport of citrulline and methionine (cytosol to extracellular)
  2. glyoxylate + L-methionine = 4-methylsulfanyl-2-oxobutanoate + glycine
  3. 1,2-dihydroxy-5-(methylsulfanyl)pent-1-en-3-one + O2 = 4-methylsulfanyl-2-oxobutanoate + formate + 2 H(+)

  Catalysed by SLC7A6, AADAT, ADI1extracellular space, cytosol

• taurineReach from here →
  1. transport of citrulline and lysine (cytosol to extracellular)
  2. transport of chloride and lysine (cytosol to extracellular)
  3. Neurotransmitter:Sodium Symporter (Nss) Tcdb:2.A.22.3.3

  Catalysed by SLC7A5, SLC7A6, SLC6A14, SLC6A6extracellular space, cytosolOne of 5 routes

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

  Catalysed by SLC7A6, MOCOS, RETSATextracellular space, cytosol

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

  Catalysed by SLC7A6, MOCOS, DIO1, DIO2extracellular space, cytosolOne of 8 routes

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

  Catalysed by SLC7A6, MOCOS, DIO1, DIO3extracellular space, cytosol

• a 3-acylpyruvateReach from here →
  1. transport of alanine and citrulline (cytosol to extracellular)
  2. (R)-3-Amino-2-methylpropanoate:pyruvate aminotransferase
  3. a 3-acylpyruvate + H2O = a carboxylate + pyruvate + H(+)

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

• (R)-methylmalonyl-CoAReach from here →
  1. transport of citrulline and lysine (cytosol to extracellular)
  2. Acetylation of Lysine
  3. aldehyde dehydrogenase (2-methyl-3-oxopropanoate)

  Catalysed by SLC7A5, SLC7A6, ALDH6A1extracellular space, cytosol, mitochondrial matrixOne of 2 routes

• (S)-methylmalonyl-CoAReach from here →
  1. transport of citrulline and lysine (cytosol to extracellular)
  2. Acetylation of Lysine
  3. (S)-methylmalonyl-CoA hydrolase

  Catalysed by SLC7A5, SLC7A6extracellular space, cytosol, mitochondrial matrix

• 3'-dephospho-CoAReach from here →
  1. transport of citrulline and lysine (cytosol to extracellular)
  2. Acetylation of Lysine
  3. acid phosphatase 2, lysosomal (CoA)

  Catalysed by SLC7A5, SLC7A6, ACP2extracellular space, cytosol, mitochondrial matrix, lysosomeOne of 2 routes

• (2E)-dodecenoyl-CoAReach from here →
  1. transport of citrulline and lysine (cytosol to extracellular)
  2. Acetylation of Lysine
  3. (2E)-dodecenoyl-CoA formation

  Catalysed by SLC7A5, SLC7A6extracellular space, cytosol, mitochondrial matrixOne of 3 routes